Neurodevelopmental evaluation of children with cyanotic congenital heart disease.

BACKGROUND: The aim of the study was to perform a neurodevelopmental evaluation of the children with cyanotic congenital heart disease and to determine the factors that affect the neurodevelopmental status. METHODS: The study was performed in the Pediatric Cardiology Department of Behcet Uz Children's Hospital between February and August 2013. Children between the age of six to forty-two months were included in the study and were evaluated in three groups (two patient groups and the control group). In group A, patients with isolated cyanotic congenital heart disease were enrolled. Group B consisted of the patients with cyanotic congenital heart disease with other concomitant diseases. Group C included the healthy control group. For the neurodevelopmental evaluation Bayley Scale of Infant Development- II (BSID-II) was used. Mental Developmental Index (MDI) and Psychmotor Developmental Index (PDI) scores were calculated. Factors possibly effective on neurodevelopment were evaluated. RESULTS: Thirty eight patients (32 in group A and 6 in group B) and 33 healthy subjects in group C were included in the study. Mean age of the patient group was 22.5±11.2 months. In group A mean MDI Score (82.5±14.7) was significantly lower than group C (92.3±6.9) (P=0.001). Similarly mean PDI Score in group A (82.0±18.2) was found significantly lower than group C (92.5±7.4) (P=0.003). When group A and B were compared, mean MDI and PDI scores were lower in group B, but the difference was not statistically significant. For group A, according to the psychomotor development index, 41.6% of the patients were found to be mildly to severely retarded. In terms of the mental development index, 34.4% of the patients had moderate or mild retardation. The sex, socioeconomic status, gestational age, birth weight, comorbidities, history of surgery and reoperation, length of stay in intensive care unit were not found influential on the MDI and PDI scores of the patients. CONCLUSIONS: Mental-motor retardation is frequently encountered in children with cyanotic congenital heart disease. These patients may benefit from motor, language, speech, developmental and educational therapies. For this reason, these children have to be under regular follow up for neurodevelopmental status.

Aminotransferase elevations in rotavirus positive and negative acute gastroenteritis and its relation with disease severity.

BACKGROUND: The aim of this study was to investigate the frequency of elevated alanine (ALT) and aspartate aminotransferase (AST) levels in children with rotavirus positive and negative gastroenteritis as well as the average time to normalization of liver enzymes. METHODS: Into the study 298 patients with rotavirus positive and 321 patients with rotavirus negative gastroenteritis were enrolled. RESULTS: Mean AST (56.9±2.1 and 40.2±0.9 U/L, respectively, P=0.000) and ALT (33.1±1.7 and 22.4±0.8 U/L, respectively, P=0.000) levels were significantly higher in the rotavirus positive than rotavirus negative patients. Logistic regression analysis showed that rotavirus positivity was significant independent factor for both AST and ALT elevation. Severity of gastroenteritis was another significant independent factor for ALT elevation. The average transaminase normalization time for AST and ALT levels were similar both rotavirus positive and negative groups. CONCLUSIONS: Rotavirus positivity and severity of gastroenteritis were independent risk factors for elevated ALT levels in children with gastroenteritis.

Asplenia in children with congenital heart disease as a cause of poor outcome.

The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. Congenital asplenia can be part of multiple congenital abnormalities as in heterotaxy including Ivemark syndrome with congenital anomalies of the heart or great vessels, or it can be isolated, which is extremely rare. In these cases, asplenia is an important factor effecting mortality. In this report, the clinical courses of five children with asplenia and concomitant minor or complex cardiac anomalies are presented. The ages of the children ranged between 1.5 and 17 months at the time of diagnosis. All of the cases had had a history of hospitalisation for infectious diseases before the diagnosis. The patient who was diagnosed at 17 months old had a history pneumonia, urinary tract infection, and bacterial meningitis beginning at five months old. Three children had complex cardiac anomalies, one child had ventricular septal defect, and one child had atrial septal defect. Howell-Jolly bodies were determined in peripheral blood smear in all of the patients. The diagnoses of asplenia were confirmed with spleen scintigraphy. One of the patients with complex cardiac anomalies died a short time after diagnosis, because of cardiac failure. The rest of the four patients were vaccinated for encapsulated bacteria and were taken under antibiotic prophylaxis. These children did not need hospitalisation for infectious diseases during the follow-up period (5-40 months). In asplenic children, early diagnosis, antibiotic prophylaxis, and immunisation for encapsulated bacteria can decrease the risk of morbidity and mortality.

Comparison of sequential and standard therapy for Helicobacter pylori eradication in children and investigation of clarithromycin resistance.

AIMS: The aim of the present study was to compare the efficacy of sequential and standard triple-drug regimen for Helicobacter pylori (H pylori) eradication in children and to determine the primary resistance rate to clarithromycin. METHODS: Children with H pylori infection randomized to receive either standard regimen (n = 28) consisting of lansoprazole for 30 days, amoxicillin and clarithromycin for 14 days or sequential regimen (n = 16) consisting of lansoprazole for 30 days, amoxicillin for 7 days, followed by clarithromycin and metronidazole for the next 7 days. Clarithromycin susceptibility of H pylori was assessed with fluorescence in-situ hybridization technique. Eradication was controlled by C urea breath test or monoclonal stool antigen test 4 weeks after the end of the therapy. RESULTS: H pylori eradication rate was higher in the sequential therapy group (93.7%), compared with the standard therapy group (46.4%) (P = 0.002). There was no difference in adverse drug reactions and in compliance to the treatment between the groups. Primary clarithromycin resistance rate for H pylori was found as 25.7% (n = 9). All of the patients having clarithromycin resistance were coincidentally in the standard therapy group. After the exclusion of these 9 patients, sequential therapy was again found to be more effective than the standard therapy (P = 0.02). CONCLUSIONS: Sequential therapy seems highly effective for eradicating H pylori in children; however, the difference between 2 groups in resistant strains was the limitation of the study. Our country needs to reassess the effectiveness of standard triple therapy regimen for H pylori eradication.

The treatment of functional constipation significantly increased quality of life in children aged 4-17 years.

BACKGROUND/AIMS: In this study, it was aimed to investigate the effect of 6-week treatment on quality of life in 4-17-year-old children with functional constipation. MATERIALS AND METHODS: The children 4-17 years old diagnosed as functional constipation according to Rome IV criteria, between June and December 2018 were included in the study. KINDL scales were applied to patients and their parents before starting treatment for 6 weeks and after the end of the treatment. RESULTS: The study was completed with 42 patients. The majority of the patients (54,8%) were female cases and the general part of them (45,2%) were between 4-6 years old. The age at onset of constipation was found to be 5,19±3,658 years. Total KINDL scores of both children and parents were lower before the treatment. Significant improvement was observed in the symptoms of constipation after 6 weeks of treatment. There was a statistically significant (p<0,05) increase in children's subgroup and total KINDL scores after treatment. Parental subgroup and total KINDL scores also showed a statistically significant (p<0,05) increase after treatment. The treatment of functional constipation was found to be highly effective with the eyes of both children and parents in improving the quality of life. (Eta value, η2. >0,25) Conclusion: A significant increase was observed in all areas of life quality and total score in children with functional constipation after a regular treatment. We think that the treatment of children with functional constipation will significantly improve their quality of life.

Retrospective evaluation of childhood paraphenylenediamine intoxication due to black henna.

BACKGROUND AND OBJECTIVES: Paraphenylenediamine (PPD) is a toxic substance in henna. Oral intake of this substance causes severe systemic toxicity. To the best of our knowledge there are no studies in the literature conducted only on children exposed to henna intoxication. METHODS: Twenty-three patients aged between 1 and 17 who referred to Nyala-Sudan Turkey Training and Research Hospital between May 2015 and June 2018 were evaluated retrospectively in terms of demographic, clinic and laboratory characteristics. RESULTS: Four (17.39%) patients were male and 19 (82.61%) were female. Average age of patients was 10.95 ± 3.2. Most of the referrals to the hospital following PPD intoxication occurred in the first 16 hours. All of the patients between 10 and 17 had taken henna for suicidal purposes. All patients had vomiting and agitation. The most common symptoms apart from these were gastrointestinal symptoms, tachycardia, tachypnea and dyspnea. Twelve (52.17%) patients had elevated liver function tests and 3 (13.04%) had developed renal failure. None of the patients had neurological complications. Two (8.70%) patients developed a need for tracheostomy. Average hospitalization period of patients was 8.5 days. Two patients died. One was in 1-5 age group and died due to renal complications, while the other was in 6-10 age group and died due to hepatic failure. CONCLUSION: PPD intoxication is a life-threatening situation even in low doses. For this reason, even asymptomatic cases should undergo physical examination and should be followed closely in terms of respiratory tract obstruction. Ensuring hydration and diuresis in the early period, steroid and adrenalin therapy for prophylaxis in terms of respiratory tract obstruction are important and tracheostomy should not be abstained in necessary cases. It should not be forgotten that symptomatic treatment for organ systems and dialysis will decrease mortality and morbidity.

A rare presentation of hydatid cyst in a child.

Cyst hydatid is the most widespread, serious cestode infection in the world. The most common organ affected by hydatid disease is the liver followed by the lungs and the two organs are affected simultaneously in about 5-13% of cases. The involvement of kidneys are rarely reported. Here we reported a patient with hydatid cysts in lung and kidney which the surgery was effective for both organs. The rarity of this case is the unusual combination of the cyst development in these organs without the involvement of liver.

Neurodevelopmental Outcome of Children with Congenital Hypothyroidism Diagnosed in a National Screening Program in Turkey.

OBJECTIVE: To study the factors affecting a neurodevelopmental status of children with congenital hypothyroidism, diagnosed on national screening program. METHODS: The study was performed in the Pediatric Endocrinology Department of Dr. Behcet Uz Children's Hospital between May 2012 and May 2013. Children with congenital hypothyroidism, aged between 24 and 36 months, diagnosed by national screening program were included in the study group. Healthy subjects at the same age group consisted of the control group. For the neurodevelopmental evaluation, Bayley Scale of Infant Development- II (BSID-II) was used. Factors possibly effective on neurodevelopment were evaluated. RESULTS: 42 patients and 40 healthy children (mean (SD) age, 29.4 (3.7) and 29.2 (3.5), respectively were included in the study. The mean MDI score [92.6 (7.07) vs 97.1 (9.69), P=0.14)] and the mean PDI score [97.8 (15.68) vs 99.1 (10.57), P=0.66)] in the study group and control group were not significantly different. Among the patient, 4.6% and 4.7% children were moderately retarded as per the MDI scores and PPI scores, respectively. The sex, socioeconomic status, birth weight, screening levels of TSH, severity of the congenital hypothyroidism, initiation time and the dosage of thyroid hormone replacement, length of the normalization period of TSH, and adherence to treatment were not found to affect the MDI and PDI scores of the patients. CONCLUSION: Some children with congenital hypothyrodism may have mild to moderate neurodevelopmental retardation, despite the early diagnosis and treatment, and thus need to be under regular follow-up for neurodevelopmental status.

Multisystemic leukocytoclastic vasculitis affecting the central nervous system.

Leukocytoclastic vasculitis is a self-limiting disease with rare systemic complications in the majority of patients. This report describes a 10-year-old male with leukocytoclastic vasculitis caused by an insect bite who presented with central nervous system, lung, kidney, and gastrointestinal involvement in addition to cutaneous findings. The diagnosis was confirmed by histopathologic examination of cerebral hematoma material. Initially, his symptoms improved with a treatment protocol including steroid, cyclophosphamide, intravenous immunoglobulin, and plasmapheresis, but the patient succumbed because of nosocomial sepsis.

Helicobacter pylori infection among children with Phenylketonuria.

This study aimed to determine the frequency of Helicobacter pylori infections in children with phenylketonuria (PKU). Sixty-six children with PKU (35 boys, 31 girls; mean age, 8.2 ± 6.7 years) and 32 outpatient controls (15 boys, 17 girls; mean age, 9.6 ± 4.7 years) were studied. Socioeconomic factors did not differ between the two groups. The frequency of H. pylori infections was higher in patients with PKU (28.1%) than in healthy controls (9.4%). In particular, a higher frequency of infection was detected in patients with PKU with poor metabolic control (51.8%). The frequency of H. pylori infection in patients with PKU with good metabolic control was only 10.2%. There was no difference in the mean total WISC-R score between the poor and good metabolic control groups. A high frequency of H. pylori infection in children with PKU with poor metabolic control could be related to many factors. Advanced and standardized clinical studies on H. pylori infections in children with PKU are required.

Hormones and cytokines in childhood obesity.

OBJECTIVE: Obesity is a growing worldwide health problem affecting both adults and children. Effective prevention and treatment modalities can be achieved by understanding the pathogenesis of obesity better. This review addresses some of the issues related to the hormones and cytokines taking part in the pathogenesis of obesity, energy balance and inflammation. DESIGN: We reviewed current literature on this broad subject especially concentrating on the functions of the hormones and cytokines taking part in the pathogenesis of the childhood obesity. Using the key words obesity, children, hormones, cytokines publications and cross references were evaluated from PubMed database between 1957 and 2009. RESULTS: In children, leptin and ghrelin are two hormones which have major influence on energy balance. Leptin is responsible from long term regulation of energy balance and ghrelin functions as an appetite stimulatory signal. In contrast to ghrelin, obestatin acts as an anorexigenic hormone by suppressing food intake. Adipokines secreted from adipose tissue are the key regulators of inflammation in obesity. Increased TNF-alpha and IL-6 levels but decreased levels of adiponectin and IL-10 are associated with increased inflammation, tissue injury and complications of obesity. CONCLUSIONS: Development, pathogenesis and complications of childhood obesity consist of complex mechanisms including numerous cytokines and hormones. New treatment modalities depend on understanding these complex mechanisms.