PURPOSE: We aimed to analyze the effectiveness and safety of low-dose midazolam and ketamine combination for upper gastrointestinal endoscopy (UGIE) in children. METHODS: The study included the children (n=425, 10.78+/-3.81 years) who underwent UGIE for diagnostic purpose during 1 year period. All children were sedated with low dose midazolam (0.1 mg/kg) and ketamine (0.5 mg/kg) intravenously. Effectiveness of the sedation and complications during the procedure and recovery period were recorded. RESULTS: Endoscopic procedure was successfully completed in 414 patients (97.4%; 95% confidence interval, 95.8-98.9). Mean+/-standard deviation (SD) duration of procedure was 6.36+/-1.64 minutes (median, 6.0 minutes; range, 4-12 minutes). Minor complications occurred during the procedure in 39.2% of the patients. The most common complication was increased oral secretion (33.1%). No major complications were observed in any patient. Age and Ramsay sedation scores of patients with complications during the procedure were lower than the others (9.49+/-4.05 years vs. 11.61+/-3.43 years, p=0.002 and 2.10+/-1.46 vs. 4.37+/-1.16, p=0.001). Mean recovery time was 22 minutes (range, 10-90 minutes; mean+/-SD, 25+/-12.32 minutes). Minor complications developed during recovery in 60.1% of the patients. The most common complication was transient double vision (n=127, 30.7%). Emergence reaction was observed in 5 patients (1.2%). CONCLUSION: The procedure was completed with high level of success without any major complication in our study. Combination of low-dose midazolam and ketamine is a suitable sedation protocol for pediatric endoscopists in UGIE.
Child* ; Conscious Sedation ; Diplopia ; Endoscopy ; Endoscopy, Gastrointestinal* ; Humans ; Ketamine* ; Midazolam*

We report a pediatric patient admitted with abdominal pain, diffuse lower extremity edema and watery diarrhea for two months. Laboratory findings including complete blood count, serum albumin, lipid and immunoglobulin levels were compatible with protein losing enteropathy. Colonoscopic examination revealed diffuse ulcers with smooth raised edge (like "punched out holes") in the colon and terminal ileum. Histopathological examination showed active colitis, ulcerations and inclusion bodies. Immunostaining for cytomegalovirus was positive. Despite supportive management, antiviral therapy, the clinical condition of the patient worsened and developed disseminated cytomegalovirus infection and the patient died. Protein losing enteropathy and disseminated cytomegalovirus infection a presenting of feature in steroid-naive patient with inflammatory bowel disease is very rare. Hypogammaglobulinemia associated with protein losing enteropathy in Crohn's disease may predispose the cytomegalovirus infection in previously healthy children.
Abdominal Pain ; Agammaglobulinemia ; Blood Cell Count ; Child ; Colitis ; Colon ; Crohn Disease* ; Cytomegalovirus ; Cytomegalovirus Infections* ; Diarrhea ; Edema ; Humans ; Ileum ; Immunoglobulins ; Inclusion Bodies ; Inflammatory Bowel Diseases ; Lower Extremity ; Protein-Losing Enteropathies* ; Serum Albumin ; Ulcer

BACKGROUND/AIMS: Isolated cecal necrosis is a rare cause of the surgical abdomen. Its manifestation is similar to that of acute appendicitis. Thirteen cases, who were pre-diagnosed with acute abdomen and were finally diagnosed with isolated cecal necrosis after operation have been evaluated alongside with literature. METHODS: The records of 13 patients, who had isolated cecal necroses between 1995 and 2011 at Necmettin Erbakan University Meram Medical School's General Surgery Clinic (Turkey), were retrospectively evaluated. RESULTS: Eight of the patients were male, whereas 5 were female. Their mean age was 68.0+/-11.7 (range 51-84) years. All the patients had at least one accompanying disease the most frequent of which were heart failure and chronic renal failure. Ten patients had right hemicolectomy and ileotransversostomy, two had right hemicolectomy and ileostomy, and one had wedge resection to the cecum by the help of linear stapler. Mortality was seen in 5 patients (38%) in the early postoperative period. CONCLUSIONS: Isolated cecal necrosis should be considered in elderly patients with chronic diseases presenting with sudden right lower quadrant pains in the differential diagnosis. Isolated cecal necrosis may have a bad prognosis since it is seen in elderly patients with accompanying problems. Therefore, early diagnosis and immediate surgical management if necessary is important to reduce the risk of morbidity and mortality.
Age Factors ; Aged ; Aged, 80 and over ; Cecum/*pathology ; Colectomy ; Female ; Heart Failure/etiology ; Humans ; Ileostomy ; Male ; Middle Aged ; Necrosis/*diagnosis/mortality/surgery ; Renal Insufficiency, Chronic/etiology ; Retrospective Studies

BACKGROUND/AIMS: Intussusception in adults is rarely seen and causes misdiagnosis due to its appearance with various clinical findings. The cause of intussusception in adults is frequently organic lesions. In this study, the underlying etiologic factors, diagnostic methods and alternative methods of treatment are discussed in the light of the literature. METHODS: In this study, a retrospective evaluation was performed on 47 cases with the diagnoses of intussusception, who were operated on for bowel obstruction between 1990-2011 in Department of Surgery of Necmettin Erbakan University Meram Medical Faculty. Data related to presentation, diagnosis, treatment and pathology were analyzed. RESULTS: Twenty-four of the patients (51%) were female, and 23 were male (49%). Mean age (year) was 49 (range: 23-78) in female group, and 50 (range: 17-72) in male group. All patients presented mechanical bowel obstruction findings and underwent operation. Intussusception was caused by benign and malignant tumors in 38 patients, and other reasons in 3 cases. No reason could be determined in the other 6 cases. Only small intestine resection was applied in 29 cases, and large intestine resection was also applied in 17 cases. Reduction and fixation surgery was performed in one patient. No postoperative mortality was observed. CONCLUSIONS: Adult intussusception remains a rare cause of abdominal pain. Diagnosis of intussusception in adults is still difficult. Main treatment was surgical in most cases.
Adolescent ; Adult ; Aged ; Colonoscopy ; Female ; Humans ; Intestinal Neoplasms/complications/diagnosis ; Intestinal Obstruction/*diagnosis/etiology/surgery ; Intussusception/*diagnosis/surgery/ultrasonography ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed ; Young Adult

Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, “the odor of sweaty feet,” abdominal pain, vomiting, feeding intolerance, shock and coma. Recurrent acute pancreatitis associated with IVA have been rarely reported. Herein; we report a child who admitted with recurrent acute pancreatic attacks and had the final diagnosis of IVA. Mutation analysis revealed a novel homozygous mutation of (p.E117K [c.349G>A]) in the IVA gene. Organic acidemias must kept in mind in the differential diagnosis of recurrent acute pancreatic attacks in children.
Abdominal Pain ; Acid-Base Equilibrium ; Acidosis ; Child* ; Coma ; Diagnosis ; Diagnosis, Differential ; Humans ; Hyperammonemia ; Hypoglycemia ; Isovaleryl-CoA Dehydrogenase ; Ketosis ; Metabolic Diseases ; Metabolism ; Odors ; Pancreatitis* ; Shock ; Vomiting

PURPOSE: Hepatopulmonary syndrome is an arterial oxygenation disorder brought about by advanced liver failure and pulmonary vascular dilatations. The reason why hypoxia develops in hepatopulmonary syndrome depends on the broadening of perialveolar capillary veins. Our study aims to investigate the effects of Flavanoid on hepatopulmonary syndrome through its inhibition of nitric oxide. METHODS: Three groups, each having 8 rats, were formed within the scope of our study. Group I (the control group) only received laparatomy, group II received choledoch ligation, and group III was administered Flavanoid (90% flavonoid diosmin, 10% flavonoid hesperidin) following choledoch ligation. The rats were administered Flavanoid at week two following choledoch ligation. The rats' livers and lungs were examined histopathologically following a five-week follow-up and the perialveolar vein diameters were measured. Arterial blood gases and biochemical parameters were evaluated. RESULTS: It was seen that fibrosis and oxidative damage in the liver with obstructive jaundice as well as hypoxia with pulmonary perialveolar vein sizes were significantly lower than the other group with cirrhosis formed through the administration of Flavanoid. CONCLUSION: We have concluded that Flavanoid administration might be useful in the treatment of hypoxia in hepatopulmonary syndrome and the delay of cirrhosis contraction.
Animals ; Anoxia ; Capillaries ; Contracts ; Dilatation ; Diosmin ; Fibrosis ; Follow-Up Studies ; Gases ; Hepatopulmonary Syndrome* ; Jaundice, Obstructive ; Ligation ; Liver ; Liver Failure ; Lung ; Oxygen ; Rats ; Veins

Percutaneous device closure of atrial septal defect (ASD) is an alternative treatment to surgery. The main advantages of the percutaneous approach include avoidance of surgery, short procedure time and hospital length, in addition to comparable rates of complications. However, percutaneous device closure is associated with infrequent early and late complications including device embolization, air embolism, cardiac tamponade and thrombotic complications. We report a rare complication of silent and late device embolization of the ASD occluder device into the right pulmonary artery, three months after implantation.
Cardiac Tamponade ; Embolism, Air ; Heart Septal Defects, Atrial ; Pulmonary Artery ; Pulmonary Embolism ; Septal Occluder Device

PURPOSE: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD.METHODS: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25).RESULTS: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term.CONCLUSION: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.
Child ; Demography ; Fibrosis ; Follow-Up Studies ; Gastroenterology ; Genetics ; Genotype ; Hepatitis, Chronic ; Humans ; Liver Diseases ; Liver ; Pathology ; Prevalence ; Prognosis

Purpose: At the beginning of the Coronavirus disease (COVID-19) epidemic, physicians paid close attention to children with chronic diseases to prevent transmission or a severe course of infection. We aimed to measure the severe acute respiratory syndrome coronavirus 2 (SARSCoV-2) antibody levels in children with chronic gastrointestinal and liver diseases to analyze the risk factors for infection and its interaction with their primary disease. Methods: This cross-sectional study analyzed SARS-CoV-2 antibody levels in patients with gastrointestinal and liver diseases (n=141) and in healthy children (n=48) between January and February 2021. Results: During the pandemic, 10 patients (7%) and 1 child (2%) had confirmed COVID-19 infection (p=0.2). The SARS-CoV-2 antibody test was positive in 36 patients (25.5%) and 11 children (22.9%) (p=0.7). SARS-CoV-2 antibody positivity was found in 20.4%, 26.6%, 33.3%, and 33.3% of patients with chronic liver diseases, chronic gastrointestinal tract diseases, cystic fibrosis, and liver transplantation recipients, respectively (p>0.05, patients vs. healthy children). Risk factors for SARS-CoV-2 antibody positivity were COVID-19-related symptoms (47.2% vs. 14.2%, p=0.00004) and close contact with SARS-CoV-2 polymerase chain reaction-positive patients (69.4% vs. 9%, p<0.00001). The use, number, and type of immunosuppressants and primary diagnosis were not associated with SARS-CoV-2 antibody positivity. The frequency of disease activation/flare was not significant in patients with (8.3%) or without (14.2%) antibody positivity (p=0.35). Conclusion SARS-CoV-2 antibodies in children with chronic gastrointestinal and liver diseases are similar to that in healthy children. Close follow-up is important to understand the long-term effects of past COVID-19 infection in these children.

Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5–204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.