Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

Helicobacter pylori infection and gastroduodenal lesions in children with chronic kidney disease stage V.

BACKGROUND: This study aimed to investigate the dyspeptic symptoms, endoscopic findings, and frequency of Helicobacter pylori (Hp) infection in children with chronic kidney disease (CKD) stage V, and to compare findings in peritoneal dialysis (PD) and hemodialysis (HD) patients. METHODS: Sixty-five patients on PD (n = 36) or HD (n = 29) were included. Age, gender, duration and type of dialysis, and dyspeptic complaints were recorded. All patients underwent endoscopy. Rapid urease tests were performed in all patients and antral biopsy examinations done in suitable patients to investigate presence of Hp infection. RESULTS: The mean age of patients (55 % male) was 13.9 ± 3.6 years. Frequency of dyspepsia was 43 % and was similar in HD and PD groups. The most frequent dyspeptic symptoms were early satiety (21.5 %) and bloating (17 %). Abnormal endoscopic findings were present in 81.5 % of patients (similar in both groups), and the most common lesion was gastritis (35.5 %). Hp positivity was determined in 37 % of the patients, which was similar in both groups. No significant relationship was found between dyspeptic symptoms and Hp infection. Hp infection was found to be significantly higher in 41.5 % of the patients with gastroduodenal lesions. Abnormal endoscopic findings were significantly higher in severely dyspeptic patients (88.9 %). CONCLUSIONS: We think performing an upper gastrointestinal tract examination and Hp screening may be helpful in renal transplant candidates with severe dyspeptic symptoms.

Value of the Likert dyspepsia scale in differentiation of functional and organic dyspepsia in children.

AIM: Dyspeptic symptoms may not allow clinicians to differentiate organic and functional gastrointestinal disorders. According to our dyspeptic patients' answers to dyspepsia questionnaire, we aimed to define the symptom scores directing organic dyspepsia (OD) before upper gastrointestinal endoscopy. PATIENTS AND METHODS: One hundred sixty-one patients (ages 10-17 years, mean 13.5 ± 2.3 years, male/female: 2/3) with chronic upper gastrointestinal system symptoms lasting for at least 3 months were enrolled. Patients with predominated reflux symptoms were excluded by 24-hour pH monitoring. Before upper gastrointestinal endoscopy, severity and incidence of 8 gastrointestinal symptoms (epigastric pain, upper abdominal discomfort, retrosternal pyrosis, bitter or sour taste in mouth, halitosis, belching, nausea, and early satiety) were measured by 5-point Likert scale. Total score indicated severity score multiplied by incidence score. Antral biopsy samples were obtained. OD is defined as peptic ulcer, erosive esophagitis, erosive or nodular gastritis, and erosive duodenitis in endoscopy and/or moderate to severe antral gastritis in histology. Functional dyspepsia (FD) is defined as normal findings/mucosal hyperemia in endoscopy and/or mild antral gastritis in antral histology. We evaluated the relation among severity and incidence scores of each dyspeptic symptom in patients with OD or FD. Age, sex, body mass index, drug history, nutritional habits, the quality of life related to dyspepsia were also investigated in patients with OD and FD. RESULTS: According to patients' histological and endoscopic findings, 100 (62%) patients were in the OD group and 61 (38%) patients were in the FD group. Of the dyspeptic complaints, the severity, incidence, and total scores of epigastric pain were significantly correlated with dyspepsia type (respectively, P = 0.042, P = 0.028, and P = 0.005). Of 93 patients who had an epigastric pain severity of 4 and 5 (namely, moderate to severe pain), 65 (70%) patients were in the OD group and 28 (30%) patients were in the FD group. Of 68 patients who had an epigastric pain severity of 0 to 3 (no epigastric pain or mild pain), 33 (48.5%) were in the OD group and 35 (51.5%) were in the FD group, and the difference was statistically significant (P = 0.042). After analyzing the total scores of 8 dyspeptic symptoms, one by one or in different combinations, we could not find a threshold (cutoff) score value that was able to indicate OD definitely. Age, sex, body mass index, and nutritional habits were not significantly different between patients with OD or FD. Nocturnal abdominal pain, pain before meals, and resolution of symptoms after meals or ingestion of antacid drugs were not significantly related to OD. Nocturnal abdominal pain was observed to be higher in the group with moderate to severe gastric inflammation. CONCLUSIONS: In the present study, the severity, incidence, and total scores of epigastric pain were significantly related to OD; however, a cutoff value of dyspepsia symptom score for differentiation of OD and FD could not determined. In our study, Likert dyspepsia scale was not beneficial in differentiation of the OD/FD groups. We suggest that the Likert dyspepsia scale should be redesigned for children or the same scale should be applied in a larger cohort of dyspeptic children.

Fecal calprotectin levels in Helicobacter pylori gastritis in children.

BACKGROUND: Fecal calprotectin is an important inflammatory marker in intestinal diseases and is not routinely used in the upper gastrointestinal system disorders. The aim of this study was to show whether there is a relationship between fecal calprotectin levels and Helicobacter pylori (H pylori) gastritis in children and to determine the association of fecal calprotectin levels with gastric biopsy results in terms of chronic inflammation and neutrophil activity. METHODS: Patients with the complaints of the upper gastrointestinal system (epigastric pain, heartburn, nausea and vomiting) who were planned to undergo endoscopy were enrolled prospectively. The presence of H pylori was defined according to the gastric antrum biopsy results. Fecal calprotectin level was tested in the stool sample of the patients. The fecal calprotectin levels, upper gastrointestinal endoscopy and gastric biopsy results of 89 patients were evaluated. RESULTS: H pylori was found to be positive in the gastric biopsies of 51 (57.3%) patients. In the H pylori positive group mean fecal calprotectin level was 74.8 ± 67 µg/g, and in the H pylori negative group mean fecal calprotectin level was 52.7 ± 46 µg/g and the difference was significant (p= 0.039). We also found a significant relationship between fecal calprotectin levels and gastric neutrophil activity grades (p= 0.034). CONCLUSIONS: Mean fecal calprotectin levels were found to be higher in H pylori positive subjects in our study. Fecal calprotectin levels were correlated with gastric neutrophil activity grades. Fecal calprotectin represents gastric neutrophilic inflammation. When interpreting a high fecal calprotectin level, H pylori infection should be kept in mind.

Long term follow-up of glomerular and tubular functions in liver transplanted patients with Wilson's disease.

The aim of this study was to determine the long term outcome of renal glomerular and tubular functions in children receiving an LT for WD. Renal functions were examined in nine children with WD before and long after LT and compared with those of nine liver transplanted children with hepatic diseases other than WD. The duration of follow-up was at least two yr for both groups. GFR, fractional TRP and tubular maximum rate of phosphate reabsorption in relation to GFR (TP/GFR) as well as daily protein and Ca excretion were studied in both groups before and after LT. Pretransplant mean GFR, TRP and TP/GFR were significantly lower in the study group than the controls. A significant increase in the post-transplant TRP and TP/GFR was observed in the study group and the difference between the groups disappeared during the long term follow-up. Urinary protein excretion decreased in both groups after LT. Tubular dysfunction is frequent in patients with WD. LT for hepatic failure secondary to WD is a lifesaving procedure correcting the underlying hepatic defect as well as renal defects.

Neurologic complications of liver transplantation in pediatric patients with the hepatic form of Wilson's disease.

The literature contains very little documentation on neurologic complications in liver transplant recipients for Wilson's disease. We retrospectively reviewed 17 consecutive cases of pediatric liver transplantation for the hepatic form of Wilson's disease to assess the types of neurologic complications that occurred, the incidence of those problems, and associated factors in this patient group. The patients were 12 boys and 5 girls; indications for liver transplantation were fulminant hepatic failure in 3 patients and chronic hepatic failure in 14 patients. Neurologic complications were observed in 10 of the 17 patients as 16 episodes. The most common neurologic complications were seizure (7 episodes in 6 patients) and sudden-onset headache (5 episodes in 4 patients). Tacrolimus was identified as the only possible cause of headache in 3 episodes. Encephalitis was the cause in 1 and intracranial hemorrhage was the cause in the other headache episode. We also noted 1 episode of tremor, 1 episode of acute dystonic reaction, 1 episode of diffuse encephalopathy, and 1 episode of common peroneal nerve palsy. Immunosuppressive agents were the primary cause of 12 of the 16 episodes of neurologic complications. Uremia with hypertension, compression of the right common peroneal nerve, encephalitis, and intracranial hemorrhages attributable to coagulopathy caused 1 neurologic episode each. Neurologic complications in patients with the hepatic form of Wilson's disease were frequent during the first 30 days after pediatric liver transplantation but did not affect survival. Transplantation teams should be aware of the high incidence of neurologic complications in pediatric patients with the hepatic form of Wilson's disease.

Ménétrier's disease and severe gastric ulcers associated with cytomegalovirus infection in an immunocompetent child: a case report.

In pediatric patients, Ménétrier's disease is an uncommon clinical entity that has been rarely described only as sporadic cases, and the etiology is unclear. These patients usually have a self-limiting clinical course. Cytomegalovirus is an important pathogen in the immunocompromised host. However, cytomegalovirus infection can be detected in non-immunocompromised children. We discuss the possible role of cytomegalovirus infection in both Menetrier's disease and severe gastric ulcers in an immunocompetent child.

Invasive esophageal aspergillosis associated with acute myelogenous leukemia: successful therapy with combination caspofungin and liposomal amphotericin B.

Aspergillosis is one of the most common invasive fungal infections in patients with leukemia. In this patient group, this form of Aspergillus infection is a life-threatening condition with a mortality of 50-100%. The lungs are most often affected, but the esophagus can also be involved.The authors report the case of a child with leukemia who developed invasive esophageal aspergillosis. The condition was diagnosed by microscopic examination of endoscopic biopsy specimens. The patient was already receiving empirical liposomal amphotericin B when the diagnosis was made, so a second antifungal (caspofungin) was added to the regimen. This combination was successful. This case to demonstrates a case of successful treatment of invasive esophageal aspergillosis using combination therapy of liposomal amphotericin B and caspofungin.

Hepatitis A super infection as a cause of liver failure in a child with Wilson's disease.

Infection with hepatitis A virus can cause severe or even fatal illness in patients with chronic liver disease. Here we describe a seven-year-old girl who presented as acute liver failure and was diagnosed with Wilson's disease and later with coexistent hepatitis A infection. Wilson's disease was demonstrated on the basis of low ceruloplasmin, high urinary copper excretion, histological evidence of cirrhosis, and high biochemical estimation of liver copper concentration. Hepatitis A was diagnosed serologically. Our case suggests that acute hepatitis A may play a part in the acute decompensation seen in some cases of unrecognized Wilson's disease. We also emphasize the importance of prevention measures of hepatitis A infection in patients with chronic liver disease.

Bocavirus Viremia and Hepatitis in an Immunocompetent Child.

BACKGROUND: So far, many studies have shown that Human Bocavirus ( HBoV) is the main pathogen of the respiratory tract. Until now, there is no study that proves the association between HBoV and hepatitis. HBoV viremia/DNAemia has been associated closely with acute primary infection and moderate-to-severe illness but, more detailed clinical data about HBoV dissemination are still unavailable. CASE REPORT: Here we report a 2-years-5-months-old girl suffering from respiratory distress and heptitis followed in our intensive care unit. HBoV was detected in our patients nose and throat swabs concurrent with whole blood sample by positive polymerase chain reactions. After a through investigation no causative agent other than HBoV viremia was found. CONCLUSION: Human Bocavirus viremia with high viral loads may be associated with hepatitis.

Neuroimaging findings in infantile GM1 gangliosidosis.

GM1 gangliosidosis is an autosomal recessive glycosphingolipid storage disease caused by defects in the enzyme beta-galactosidase. Three clinical forms (infantile-, juvenile-, and adult-onset) of the disease are recognized. Patients with infantile GM1 gangliosidosis present at birth or shortly thereafter with somatic and bony changes, followed by severe neurological deterioration ultimately leading to death within the first 2 years of life. We present the brain CT, MRI and MR spectroscopy (MRS) findings in a 17-month-old Turkish girl with infantile GM1 gangliosidosis. Neuroimaging findings in patients with infantile GM1 gangliosidosis have been reported only in a few cases. In this study, MRS of the thalamus was performed to study the metabolic changes in GM1 gangliosidosis. We showed a a decreased NAA/Cr ration and an increased Cho/Cr ratio. To our knowledge, this is the first report of magnetic resonance spectroscopy findings in type-1 GM1 gangliosidosis.

Should we routinely perform blood tests in children with uncontrolled seizures?

The value of biochemical tests in treating patients with uncontrolled seizures is unclear. We present the case of an 8-year-old boy with uncontrolled seizures receiving two antiepileptic drugs. He had been diagnosed with recurrent herpes encephalitis and treated with acyclovir 1 year previously. Laboratory blood analyses, performed because of his uncontrolled seizure episodes, revealed hypocalcemia. Hypoparathyroidism was detected with elevated levels of phosphorus and low levels of parathormone. In conclusion, blood tests, especially to measure calcium, in children with uncontrolled seizures are suggested. Hypoparathyroidism causing hypocalcemia, as present here, is not a rare occurrence.

Etiologies, outcomes, and prognostic factors of pediatric acute liver failure: A single center's experience in Turkey.

BACKGROUND/AIMS: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure. MATERIALS AND METHODS: Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2. RESULTS: There were 37 (40.6%) patients in Group 1 (spontaneous recovery) and 54 (59.4%) patients in Group 2. Thirty-two patients (35.2%) underwent liver transplantation. Infectious and indeterminate causes were the most common etiologies (33% each). Among the infectious causes, hepatitis A (76%) was the most frequent. Hepatic encephalopathy grade 3-4 on admission and during follow-up and high Pediatric Risk of Mortality (PRISM) and Pediatric End-Stage Liver Disease (PELD) scores within the first 24 h were related with a poor prognosis. Group 2 had a more prolonged prothrombin time, higher international normalized ratio, more prolonged activated partial thromboplastin time (aPTT), and higher levels of total and direct bilirubin, ammonia, and lactate (for all, p<0.01). CONCLUSION: Infectious and indeterminate cases constituted the most common etiology of PALF, and the etiology was related to the prognosis in our series. Although high PELD and PRISM scores were related to poor prognoses, no sharp thresholds for individual laboratory tests could be elucidated. Liver transplantation was the only curative treatment for patients with poor prognoses and resulted in high survival rates (1-, 5-, and 10-year survival rates of 81.3%, 81.3%, and 75%, respectively) in our study.

Does bee pollen cause to eosinophilic gastroenteropathy?

Bee pollen is given to children by mothers in order to strengthen their immune systems. There are no studies related with the side effects of bee polen in the literature. In this article, the literature was reviewed by presenting a case of allergic eosinophilic gastropathy related with bee polen. A 5-year old child was admitted due to abdominal pain. Edema was detected on the eyelids and pretibial region. In laboratory investigations, pathology was not detected in terms of hepatic and renal causes that would explain the protein loss of the patient diagnosed with hypoproteinemia and hypoalbuminemia. Urticaria was detected during the follow-up visit. When the history of the patient was deepened, it was learned that bee pollen was given to the patient every day. The total eosinophil count was found to be 1 800/mm(3). Allergic gastroenteropathy was considered because of hypereosinophilia and severe abdominal pain and endoscopy was performed. Biopsy revealed abundant eosinophils in the whole gastric mucosa. A diagnosis of allergic eosinophilic gastropathy was made. Bee polen was discontinued. Abdominal pain and edema disappeared in five days. Four weeks later, the levels of serum albumin and total eosinophil returned to normal.

Adenovirus infection as possible cause of acute liver failure in a healthy child: a case report.

Adenoviruses are common viral pathogens in childhood; however, they can cause serious disease in an immunocompromised host. Fulminant hepatitis is a rare complication of adenoviral infection. We report herein a case of fatal fulminant hepatitis possibly caused by adenovirus infection. Although rare, adenovirus infection should be considered in the differential diagnosis of acute liver failure in immunocompetent children.

Liver transplantation for hepatocellular carcinoma in children.

We present our experience with living-donor liver transplantation in the treatment of nine children with hepatocellular carcinoma. Between January 2001 and March 2007, we performed 81 liver transplantations in 79 children at our center. Nine of the 79 children (11.3%; mean age, 9.7 +/- 5.5 yr; age range, 12 months-16 yr; male-to-female ratio, 2:1) underwent an living-donor liver transplantation because of hepatocellular carcinoma. Two of nine children received right lobe grafts, three received left lateral segment grafts, and the remaining four children received a left lobe graft. According to the TNM staging system, two children had stage 1 carcinoma, three had stage 2, and four had stage 4A(1). The mean follow-up was 19.8 +/- 10.6 months (range: 7-32 months). There has been only one tumor recurrence, which occurred in the omentum 26 months after liver transplantation. There was no evidence of recurrence or AFP elevation in the other eight children. Both graft and patient survival rates are 100%. In conclusion, liver transplantation is a life-saving procedure for children with chronic liver disease with accompanying hepatocellular carcinoma. During follow-up of patients with chronic liver disease, serial AFP screening and combined radiologic imaging studies should be mandatory.

Neurological complications of liver transplantation in pediatric patients: a single center experience.

Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 +/- 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilson's disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilson's disease had a higher incidence of NCs (60%) than did patients without Wilson's disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.

Cows milk protein enteropathy and granulomatous duodenitis in a newborn.

Cow's milk protein enteropathy is a symptom complex that composed of severe diarrhoea and malnutrition. This disorder is caused by non-immunoglobulin E-mediated food allergy. Its clinical features and natural course have been explained in many reports, of different types of cow's milk and soy reactions. In the present article, we describe a newborn patient who presented with chronic diarrhoea and failure to thrive diagnosed as cow's milk protein enteropathy. The duodenal biopsy revealed granulomatous duodenitis which has not been described before. Her clinical and pathological findings responded well to cow's milk elimination. We suggest that food allergies should be considered in differential diagnosis of patients with chronic diarrhoea and failure to thrive.

Successful treatment of hepatitis B-associated leukocytoclastic vasculitis with lamivudine treatment in a child patient.

Chronic hepatitis B infection (HBI) has many extrahepatic manifestations such as vasculitis, glomerulonephritis, arthritis, dermatitis, pulmonary disease, and skin manifestations. The mechanism of these manifestations is thought to be immune mediated. Immune-suppressive treatment may enhance viral replication and worsen hepatic disease. Lamivudine is a nucleoside analogue used in chronic HBI treatment that works by suppressing replication of the hepatitis B virus (HBV). Recently, several reports have suggested that lamivudine treats vasculitis associated with HBV infection in adults. However, there are no data in the literature for children. Herein, we report a child with leukocytoclastic vasculitis due to chronic HBI who was successfully treated with lamivudine.

Hepatitis A and parvovirus B19 infections in an infant with fulminant hepatic failure.

Acute viral hepatitis with hepatitis A, B, C, D, and E viruses in the etiology of fulminant hepatic failure either single or in combinations has been described. Parvovirus B19 is also an etiologic agent of acute liver failure and hepatitis-associated aplastic anemia. We present a patient diagnosed with fulminant hepatitis A referred for liver transplantation. Parvovirus B19 superinfection was detected when the patient developed anemia during the course of the disease. We discuss possible roles of both viruses in fulminant hepatitis and pure red cell aplasia.