Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants.

BACKGROUND: Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication. CASE PRESENTATION: A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of SPINK1. We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches. CONCLUSIONS: PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy.

Lupus enteritis as the sole presenting feature of systemic lupus erythematosus: case report and review of the literature.

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune inflammatory disease which can affect any organ, including the gastrointestinal tract. Lupus enteritis is one of the manifestations of gastrointestinal involvement in SLE patients. However, it is exceedingly rare that lupus enteritis is the sole initial presentation of SLE. A 12-year-old Thai girl who had had recurrent abdominal pain for 2 months with no other signs of SLE on initial presentation is described. A single-balloon enteroscopy demonstrated segmental erythema of the proximal and mid-jejunum. Histopathology demonstrated active enteritis and submucosal vasculitis. On the basis of evidence of intestinal vasculitis, autoimmune profiles were performed; the results supported the possibility of SLE. She subsequently developed leucopenia, lymphopenia and an oral ulcer, leading to a robust diagnosis of SLE. Her clinical condition improved dramatically with prednisolone. Even though lupus enteritis is rare, it can be the initial presentation of SLE. In young adolescent girls with recurrent abdominal pain, the possibility of lupus enteritis should be borne in mind.

Impact of a group-based treatment program on adipocytokines, oxidative status, inflammatory cytokines and arterial stiffness in obese children and adolescents.

Background Dysregulation of adipocytokines, inflammatory cytokines and oxidative stress are associated with the pathogenesis of obesity-related complications. This study aimed to evaluate the effect of a group-based lifestyle modification program on adipocytokines, inflammatory cytokines, oxidative status and arterial stiffness in obese youth. Methods A 1-year weight-reduction program was conducted. The program consisted of initial hospitalization and five outpatient group-based sessions held at 1, 2, 3, 6 and 9 months. Pre- and post-intervention measurements included anthropometric data, blood tests, body composition and brachial-ankle pulse wave velocity (ba-PWV). Results A total of 126 obese youths were recruited, and 115 of those completed the study. Twenty-four participants had increased percentage weight for height at the end of the study (group A), 30 had minimal reduction (group B) and 61 had substantial reduction (group C). Lean mass significantly increased in all three groups (all p<0.001). A significant decrease in leptin (group A, p=0.021; group B, p=0.005; group C, p<0.001), interleukin-6 (IL-6) (group A, p=0.019; group B, p=0.004; group C, p<0.001) and ba-PWV (group A, p=0.031; group B, p=0.015; group C, p<0.001) was also observed. No significant change in the oxidative status was found among the groups. Reduction in ba-PWV was correlated with decreases in plasma malondialdehyde (pMDA) (r=0.233, p=0.036) and homeostasis model assessment of insulin resistance (HOMA-IR) (r=0.253, p=0.025). Conclusions A group-based healthy lifestyle program for obese youths had beneficial effects on adipocytokines, inflammatory cytokines and arterial stiffness. Participants without change in weight status also benefited. These improvements may reduce the risk of obese youths developing atherosclerosis.

Outcomes of group-based treatment program with parental involvement for the management of childhood and adolescent obesity.

OBJECTIVE: An uncontrolled study was conducted to evaluate the effects of a group-based program on weight control, metabolic profiles, and obesity-related complications in obese youth. METHODS: The program consisted of an initial in-patient session and five group sessions, one, two, three, six, and nine months into the study, providing participants and their parents with information about the consequences of obesity and lifestyle modifications. The severity of obesity and obesity-related complications were evaluated at baseline and 12 months after the intervention. The participants' and their parents' perceptions of the program were assessed. RESULTS: Of the obese youth recruited (n=126), 115 completed the study. Their percentage weight for height and percentage body fat decreased significantly (both p<0.001), and their insulin resistance, lipid profiles, and transaminases levels improved (all p<0.01). The prevalence of prediabetes, dyslipidemia, and elevated transaminases decreased significantly (all p<0.05). The participants and their parents perceived the program as valuable. CONCLUSION: A group-based program is effective in managing childhood obesity, improving metabolic profiles, and alleviating certain obesity-related complications. PRACTICE IMPLICATIONS: A group-based program that provides education and raises the awareness of obese children and their parents about the consequences of obesity is an effective model for treating childhood obesity.

Clinical effectiveness of an anesthesiologist-administered intravenous sedation outside of the main operating room for pediatric upper gastrointestinal endoscopy in Thailand.

Objectives. To review our sedation practice and to evaluate the clinical effectiveness of an anesthesiologist-administered intravenous sedation outside of the main operating room for pediatric upper gastrointestinal endoscopy (UGIE) in Thailand. Subjects and Methods. We undertook a retrospective review of the sedation service records of pediatric patients who underwent UGIE. All endoscopies were performed by a pediatric gastroenterologist. All sedation was administered by staff anesthesiologist or anesthetic personnel. Results. A total of 168 patients (94 boys and 74 girls), with age from 4 months to 12 years, underwent 176 UGIE procedures. Of these, 142 UGIE procedures were performed with intravenous sedation (IVS). The mean sedation time was 23.2 +/- 10.0 minutes. Propofol was the most common sedative drugs used. Mean dose of propofol, midazolam and fentanyl was 10.0 +/- 7.5 mg/kg/hr, 0.2 +/- 0.2 mg/kg/hr, and 2.5 +/- 1.2 mcg/kg/hr, respectively. Complications relatively occurred frequently. All sedations were successful. However, two patients became more deeply than intended and required unplanned endotracheal intubation. Conclusion. The study shows the clinical effectiveness of an anesthesiologist-administered IVS outside of the main operating room for pediatric UGIE in Thailand. All complications are relatively high. We recommend the use of more sensitive equipments such as end tidal CO(2) and carefully select more appropriate patients.

Experience of intravenous sedation for pediatric gastrointestinal endoscopy in a large tertiary referral center in a developing country.

BACKGROUND: The aim of this study was to evaluate the clinical efficacy of intravenous sedation for pediatric gastrointestinal endoscopy (GIE) at a tertiary care teaching hospital in a developing country. METHODS: We undertook a retrospective review of the sedation service records of pediatric patients who underwent GIE. All endoscopies were performed by a pediatric gastroenterologist. All of the sedation was administered by staff anesthesiologist or anesthetic personnel in the gastroenterology procedure room. RESULTS: Sedation was provided for 222 procedures in 214 patients ranged in age from younger than 1 to 17 years and in weight from 2.7 to 80.0 kg. Intravenous sedation was provided in 176 patients (82.2%). Of these patients, 185 procedures were performed and reviewed, with 152 (82.2%) procedures were esophagogastroduodenoscopy (EGD) alone, 14 (7.6%) procedures were colonoscopy alone, 18 (9.7%) procedures were EGD and colonoscopy, and one procedure was endoscopic ultrasonography (EUS). Most common indications of the procedure were screening for esophageal varices (25.2%), abdominal pain (15.9%), history of upper gastrointestinal hemorrhage (13.6%), and unexplained anemia (10.3%). The majority of preanesthetic problems were hematologic disease, anemia (38.2%); liver disease, cirrhosis (13.5%); and electrolyte imbalance (13.5%). Propofol (94.0%), fentanyl (87.0%), and midazolam (67.8%) were frequently used. The mean dose of propofol was 7.8 +/- 4.1 mg.kg(-1).h(-1), fentanyl 2.3 +/- 1.1 mcg.kg(-1).h(-1), and midazolam 0.1 +/- 0.1 mg.kg(-1).h(-1). Most of them were used in combination. The combination of propofol, fentanyl, and midazolam was commonly employed (46.4%). The mean sedation time of all procedures was 28.2 min and was different according to procedure type. Complications occurred infrequently (13.5%) and were medication or airway related. All complications were easily treated, with no adverse sequelae. Intravenous sedation was successful except for one patient who required general anesthesia. However, all procedures were completed successfully. CONCLUSIONS: In the setting of the developing country, intravenous sedation for pediatric GIE by trained anesthetic personnel with appropriate monitoring was safe and effective. Serious adverse events were rare in our population.

Severe congenital systemic juvenile xanthogranuloma in monozygotic twins.

Juvenile xanthogranuloma, a histiocyte disorder, usually presents with a solitary cutaneous lesion. Juvenile xanthogranuloma with extracutaneous involvement is a rare disease in which significant morbidity and occasional deaths may occur. Monozygotic twins with congenital systemic juvenile xanthogranuloma who presented with multiple skin lesions, hepatosplenomegaly, liver failure, and bone marrow involvement were reported. The diagnosis of systemic juvenile xanthogranuloma was confirmed by histology and immunohistochemical stains of the skin with liver biopsies revealing dense infiltration of lymphohistiocytes with typical Touton giant cells staining positive for CD68 and negative for CD1a and S-100 protein. Both of them received systemic prednisolone 1 mg/kg/day which was gradually tapered off with time according to clinical and investigative responses. At the 17-month follow-up period, both patients showed remarkable regression in all symptoms and laboratory studies.

Anterior pituitary hormone effects on hepatic functions in infants with congenital hypopituitarism.

BACKGROUND: Congenital hypopituitarism is an uncommon cause of neonatal cholestasis. Little is known about the effect of anterior pituitary hormone on hepatic functions. METHODS: A retrospective review of the medical charts of eight infants with congenital hypopituitarism and neonatal cholestasis was performed. The results of endocrinological investigations, eye examinations, and magnetic resonance imaging were used to classify these infants. RESULTS: Eight infants (4 male and 4 female; mean age, 1.7 weeks) who presented with cholestatic jaundice subsequently (mean age, 7.6 weeks) developed isolated or multiple anterior pituitary hormone deficiencies. Persistent hypoglycemia, ocular abnormalities, and microphallus were often clinical signs prompting further endocrinological and radiological investigations. Septo-optic dysplasia was prevalent, occurring in five cases. Cholestasis and hepatosplenomegaly resolved within a mean of 9.7 and 10 weeks, respectively, in the majority of cases after replacement of glucocorticoid and thyroid hormones. However, transaminase levels remained high after hormone replacement. Cortisol deficiency and hypoglycemia were noted in all cases, often following stress. Hyperlipidemia persisted in one case after the resolution of cholestasis and after corticosteroid and thyroid hormone replacement therapy. Growth hormone deficiency was not corrected due to the absence of hypoglycemia after corticosteroid hormone, an infant's age, and/or a lack of financial resources. CONCLUSIONS: In our series, it appears that glucocorticoid and thyroid hormones play a significant role in the resolution of cholestasis and hepatosplenomegaly. A persistently elevated transaminase level and hyperlipidemia after corticosteroid and thyroid hormone replacement may indicate the need for long-term follow-up and/or growth hormone therapy.

Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: an interplay of unbalanced immune regulatory mechanisms.

Vanishing bile duct syndrome (VBDS) is a rare disorder and requires a liver biopsy for a diagnosis. The condition has not been reported in children with toxic epidermal necrolysis (TEN). The etiology of VBDS in our patient with TEN is most likely from drug hypersensitivity. A high index of suspicion will prompt clinicians to start more specific investigations and treatments. The use of immunosuppressive agents, intravenous immunoglobulin and ursodeoxycholic acid has not been consistently successful in these patients. A new approach with biologic agents such as anti-tumor necrosis factor-alpha may be a promising therapy and reduce severe adverse outcomes.

A rapid serologic test and immunoblotting for the detection of Helicobacter pylori infection in children.

The gold standard for the diagnosis of Helicobacter pylori infection requires an endoscopic biopsy of gastric mucosa for histological examination, urease test and culture. Noninvasive serological tests are useful as a screening test for H. pylori infection. The aim of this study was to evaluate the performance of a rapid office-based serologic test, using immunochromatography ICM, and the immunoblotting for the diagnosis of H. pylori infection in Thai children. Eighty-two symptomatic children, 30 boys and 52 girls (mean age 9.2+/-3.8 years; range, 1.2-16.0 years) who had no previous treatment for H. pylori underwent upper endoscopy. Biopsies were obtained from the gastric body and antrum for histopathology and rapid urease test. Serum samples collected from all patients were tested for H. pylori IgG antibodies using ICM (Assure H. pylori Rapid Test, Genelabs Diagnostics, Singapore). Immunoblotting (HelicoBlot 2.1, Genelabs Diagnostics, Singapore) was tested in sera of 75 patients to detect antibodies to specific antigens of H. pylori. Positive H. pylori status was defined as positive for both histology and rapid urease test. Of 82 patients, 25 (30.5%) were H. pylori positive, 56 (68.3%) were H. pylori negative and one was equivocal. ICM assay yielded a positive result in 24 of the 25 H. pylori-positive patients (96.0%) and 3 of the 56 H. pylori-negative patients (5.4%). The immunoblotting yielded a positive result in all of 22 H. pylori-positive patients (100%) and in 2 of the 52 H. pylori-negative patients (3.8%). Obtained ICM's sensitivity, specificity, positive predictive value, negative predictive value and accuracy were 96.0, 94.6, 88.9, 98.1 and 95.1%, with immunoblotting 100.0, 96.2, 91.6, 100.0, and 97.3%, respectively. The immunochromatographic and immunoblot tests are non-invasive, reliable and useful for the diagnosis of H. pylori infection in Thai children.

Neonatal cholestasis in Thai infants.

The objective of this study was to study etiologies and outcome of neonatal cholestasis in Thai infants. The medical records of infants aged less than 3 months with the diagnosis of neonatal cholestasis in Department of Pediatrics, Siriraj Hospital from 1993 to 2004 were retrospectively reviewed. The etiologies were diagnosed by history, physical examination, and proper investigations. There were 252 infants, including 135 males (53.6%) and 117 females (46.4%). The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. TPN-related cholestasis was increasingly found due to advance management of critically ill premature infants. Inborn error of metabolism were suspected in 8 patients (3.21%). Seventeen cases (6.7%) developed cholestasis during the first week of life due to hemolytic anemia, intrauterine infection, hypoxia and others. During the 3 month follow-up period, 6 cases died of progressive dysfunction of liver and one case with idiopathic neonatal hepatitis died from intracranial bleeding from vitamin K deficiency. In conclusion, INH and EHBA are the most common causes of neonatal cholestasis. Due to advance management and nutritional support in critically ill premature infants, TPN-related cholestasis is found more often. Inborn error of metabolism related to neonatal cholestases is uncommon in Thai infants. Overall short-term prognosis of neonatal cholestases is good.

Suggestive parameters for eradication therapy in children with Helicobacter pylori gastritis.

The relationship between Helicobacter pylori (H. pylori) infection and recurrent abdominal pain in children is still controversial. H. pylori-infected children with recurrent abdominal pain generally do not require treatment. However, benefit of treatment has been known to produce dramatic improvements in some patients. Furthermore, H. pylori-infected is associated with growth retardation, iron deficiency anemia and thrombocytopenia. The objective of this study was to find suggestive parameters for eradication of H. pylori gastritis. From 1992 to 2004, medical records of 42 children diagnosed as having H. pylori infection by endoscopy were retrospectively reviewed. Of those 42 patients, there were 36 patients with H. pylori gastritis without gastric or duodenal ulcer (85.7%), and 6 patients with ulcers (14.3%). Children with H. pylori gastritis were divided into 2 groups: responsive and unresponsive. Data including the duration of abdominal pain, endoscopic finding, histology, treatment, outcome and final diagnosis were collected. Additional data were obtained by telephone and letters. Of 36 patients, there were 24 and 12 patients in responsive and unresponsive groups, respectively. Three patients with anemia were all presented in the responsive group. Those experiencing abdominal pain less than 3 months more commonly belonged to the responsive group (P = 0.21). Marked erythema of gastric mucosa was only seen in the responsive group (P = 0.136). All cases of chronic moderate-active gastritis appeared in the responsive group (p = 0.03). In conclusion, iron deficiency anemia and chronic moderate-active gastritis should be the suggestive parameters for eradication therapy in children with H. pylori gastritis.

Hematemesis in infants induced by cow milk allergy.

This study was conducted in order to analyze the clinical manifestations, the endoscopic findings, the histology of the gastrointestinal mucosa, the treatments and the clinical course in infants who had hematemesis induced by cow milk allergy. The medical records were reviewed retrospectively. The criteria for the diagnosis of CMA included elimination of cow milk formula resulting in improvement of symptoms, specific endoscopic and histologic findings as well as the exclusion of other causes. Twenty-three infants with a diagnosis of hematemesis were analyzed, which included 20 infants with CMA and 3 infants with gastroesophageal reflux disease (GERD). In the CMA group were 12 girls and 8 boys whose ages were 4.3 +/- 1.4 months. The onset of vomiting after starting cow milk formulas was 70.6 +/- 48.9 days. Gastroduodenoscopy was performed on 15 patients showing erythema, erosion and friability of the gastric mucosa in all patients and lymphoid hyperplasia in the duodenal bulb in 7 patients. Eight patients had mild to moderate eosinophilic infiltration and 5 patients had eosinophilia. Cow milk formulas were changed to other formulas: two children were initially given extensively hydrolyzed casein formulas and later followed by a soy formula, 14 were given a soy formula and 4 were given partially whey hydrolyzed formulas. All patients showed clinical signs of improvement a few days later. Patients that were able to tolerate cow milk were 1.5 +/- 0.9 years old. During the follow-up period (2.6 +/- 1.8 years after treatment) 4 patients were diagnosed with asthma, 4 patients with chronic respiratory symptoms, 4 patients with constipation and 2 others with food allergies. CMA induced gastritis in infancy may not be classified as eosinophilic gastritis because of the low level of eosinophilic infiltration. The elimination of cow milk and subsequent substitution with a soy formula is the proper management.

Cyclic vomiting syndrome in Thai children.

Cyclic vomiting syndrome (CVS) is a severe childhood vomiting disorder of unknown etiology and pathogenesis. Clinical manifestations and prophylactic therapy of vomiting have been described in the literature. The data were limited in Asian children. The aim of this study was to study the clinical manifestation, to evaluate using antimigraine prophylactic drugs and response in Thai children with CVS. The medical records of children with a diagnosis of CVS in the Department of Pediatrics, Siriraj Hospital, Mahidol University from 1994 to 2001 were retrospectively reviewed. Demographic data, clinical manifestations, investigations, treatment and outcome were collected and analyzed. Twenty five patients were enrolled in this study including 13 females and 12 males. Their ages ranged from 2.3 years to 14 years (7.8 +/- 3.4 years). The age of onset was 5.2 +/- 3.2 years. They had 14.7 +/- 6.5 episodes per year with a duration of each attack 4 +/- 1.8 days. There were 8 mild, 10 moderate and 7 severe cases. There were only 6 patients (24%) who had headache and 50 per cent of these had a family history of migraine. Eight patients received pizotifen which had 3 good, 1 fair, and 4 poor responses. Of this group, in 3 patients pizotifen was changed to amitriptyline. Eighteen patients received amitriptyline and the result of treatments were 11 good, 4 fair, and 3 poor. The other 2 patients were on propranolol with one good and one poor responses. The efficacy of amitriptyline and pizotifen were compared (83.3% vs 50%) which revealed no statistical significance (p = 0.14). There was no side effect from any of the medication in this study. In conclusion, the present report showed similar data of clinical features, prophylactic treatment and outcome as previous reports, except for fewer migraine headaches in patients and their families. Amitriptyline and pizotifen were effective in prophylactic therapy of vomiting episodes.