Ayhan Gazi Kalayci

Cardiac Functions in Children with Vitamin D Deficiency Rickets

Abstract. Nutritional deficiency of vitamin D is common in developing countries as a result of both inadequate diet and exposure to ultraviolet light. The most striking biochemical finding in this illness is hypocalcemia. Reduction in serum calcium level may affect ventricular contraction. The purpose of this study was to evaluate prospectively left ventricular function in a group of 27 infants diagnosed as having rickets. Electrocardiograms and echocardiographic studies were undertaken in all patients. A group of ten healthy infants was used as a control for the echocardiographic examinations. Patients were divided into three groups according to the biochemical classification of rickets. There were eight patients in group I, nine in group II, and ten in group III. Abnormal electrocardiographic findings were noted in four infants in group I, three in group II, and six in group III before treatment of the rickets. These changes resolved following treatment. Echocardiographic studies revealed left ventricular dysfunction in the pretreatment stage. The most striking echocardiographic finding is the increase in the ratio of interventricular septal thickness to left ventricular posterior wall thickness in eight patients from group III. This returned to normal after treatment of the rickets. This study has demonstrated echocardiographic evidence of left ventricular dysfunction in children with rickets. These abnormalities were not, however, sufficiently severe to be associated with clinical signs of cardiac failure. Cardiomyopathy may develop in rickets, especially in the third stage of the disease, and this finding may return to normal following adequate treatment of the rickets.

Intravenous Immunoglobulin Treatment in Children with Guillain-Barre Syndrome

Guillain-Barre syndrome is an acquired demyelinating polyneuropathy that is presumed to be immune-mediated. On the basis of this assumption, intravenous immunoglobulin (IVIG) has been used in the treatment of Guillain-Barre syndrome in recent years and found to be effective. To test this we performed a randomized study in patients with Guillain-Barre syndrome by giving IVIG (1 g/kg body weight per day over 2 consecutive days) in 9 children who were compared with 9 patients who were observed but not given specific therapy. We concluded that intravenous immunoglobulin is a safe and effective treatment for childhood Guillain-Barre syndrome which shortens the time to recovery.

Acute immune thrombocytopenic purpura: A comparative study of very high oral doses of methylprednisolone and intravenously administered immune globulin☆☆☆★

We compared very high doses of methylprednisolone with intravenously administered immune globulin for treatment of acute idiopathic thrombocytopenic purpura. Fifty-seven children were randomly assigned to receive the immune globulin preparation, 0.5 gm/kg per day for 5 consecutive days (n = 19), orally administered methylprednisolone, 30 mg/kg per day for 7 days (n = 19), or orally administered methylprednisolone, 50 mg/kg per day for 7 days (n = 19). There were no differences in the response of the platelet counts among the groups. We conclude that these two therapies were equally effective; choice between them may be made according to cost and therapy-related risks.

The prevalence of coeliac disease as detected by screening in children with iron deficiency anaemia

AIM Iron deficiency anaemia is a frequent finding seen in coeliac disease, which can be diagnosed alone or with other findings. In this study, our aim was to determine the prevalence of coeliac disease in children with iron deficiency anaemia without significant gastrointestinal symptoms. METHODS There were 135 children with iron deficiency anaemia in the patient group (group 1), and 223 healthy children without iron deficiency anaemia in the control group (group 2) in this study. Antiendomysial antibody (EMA) IgA test was given to both groups. Antiendomysial antibody-positive patients underwent small intestine biopsy. RESULTS The mean age was 7.2+/-4.6 (2-16) y in the patient group (group 1) and 8.2+/-3.8 (2-16) y in the control group (group 2), and no significant difference between the two groups was detected. In terms of gender, there was a significant difference between groups 1 and 2 (M/F: 74/61 and 98/125, respectively) ( p<0.05). EMA was positive in six cases in group 1 (4.4%), and villous atrophy and/or inflammation in the lamina propria with increased intraepithelial lymphocytes was seen on small intestine biopsy in these patients. In the control group, EMA was negative in all children. In detailed histories of patients with coeliac disease diagnosis, recurrent iron deficiency anaemia/pica was found in four patients (66.7%) and occasionally foul-smelling or watery stool attacks were seen in four patients (66.7%). Three of these six patients (50%) had short stature. CONCLUSION The prevalence of coeliac disease was high in patients with iron deficiency anaemia; therefore, gastrointestinal findings should be further examined for coeliac disease, and the possibility of coeliac disease should be investigated in patients with recurrent iron deficiency anaemia and short stature.

The incidence of gallbladder stones and gallbladder function in beta-thalassemic children.

Purpose: To determine gallbladder motor function and gallstone prevalence in beta-thalassemic children. Abnormalities in gallbladder function or bile acid metabolism may contribute to gallstone formation in these patients. Material and Methods: In 17 beta-thalassemic patients and 12 normal healthy children with similar age, sex and weight, gallbladder size was measured using real-time US, and volume was calculated using the ellipsoid method. Results: In the beta-thalassemic patients, cholelithiasis was present in 2 patients (11.8%). Sludge, which can be a predisposing factor for cholelithiasis and cholecystitis when it persists, was detected in 5 patients (29.4%). One of the patients had both cholelithiasis and sludge. Compared with the control group, beta-thalassemic children had larger fasting volume, residual volume, and smaller contraction index. Conclusion: Beta-thalassemic patients have enlarged gallbladders that retain an increased residual volume of bile. Gallbladder enlargement, bile stasis, and impaired emptying of sludge may be important events in the pathogenesis of pigment gallstones in beta-thalassemic patients.

Henoch–Schönlein purpura associated with hepatitis A infection

common vasculitis conditions of childhood, and is characterized by nonthrombocytopenic purpura, arthritis and/or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal involvement. Although the etiology of HSP is still unknown, it is considered to be an immune-mediated vasculitic disorder resulting from an immune complex reaction to various antigenic stimuli. Many bacterial and viral organisms such as streptococci, adenovirus, parvovirus, hepatitis B virus (HBV), Epstein–Barr virus, varicella and mycoplasma have been reported as predisposing factors for HSP.1 So far, HSP associated with hepatitis A virus (HAV) infection has only been reported in one other patient, as a letter to the editor.2 In the present report we describe two patients with HSP associated with acute hepatitis A.

Gallbladder motility in children with Down syndrome.

Objective: The aim of this study was to investigate gallbladder motility in children with Down syndrome by measuring gall-bladder volume and contraction index. Methods: This study, performed between January 2001 and December 2002 at the Ondokuz Mayis University, School of Medicine, Department of Paediatric Neurology, Samsun, Turkey, included 21 patients with Down syndrome (study group) and 22 healthy children (control group). After an 8-hour fast, gallbladder diameters in both groups were measured in length, width, and height by ultrasonography before and 30 minutes after a test meal. The volume of gallbladder before and after a test meal was determined, and the contraction index was calculated. Blood triglyceride and cholesterol levels were measured, and 5-hydroxyindoleacetic acid (5-HIAA) levels in urine were determined. Results: Mean gallbladder volume before test meal in the study group and controls was 8,412.4 ± 5,174 mm3 and 16,516.8 ± 6,796.1 mm3 (P < 0.001), respectively. The mean contraction index of the study group was 41.2% ± 19.4% and of controls, 75.0% ± 12.3% (P < 0.001). The mean triglyceride level of the study group was significantly higher than controls (P < 0.05). The mean urine 5-HIAA level of the study group was lower than controls (P < 0.05). Conclusion: CI was lower in patients with Down syndrome, suggesting gallbladder hypomotility. Hypomotility may be a feature associated with the high prevalence of gallstones in Down syndrome.

Celiac disease in 87 children with typical and atypical symptoms in Black Sea region of Turkey

BackgroundCeliac disease presents with a spectrum of clinical disorders. The variety of clinical presentations largely depends on age and extraintestinal findings. This study aimed to determine typical and atypical cases according to presenting symptoms and to evaluate their biochemical and pathological parameters.MethodsEighty-seven patients with celiac disease in our unit between 2000 and 2007 were reviewed. Their diagnosis was made by serological and histological examination. The patients were divided into two groups according to their typical or atypical symptoms.ResultsThe mean age of the patients at diagnosis was 8.2 years (range, 1–18 years), but patients presenting with typical symptoms were younger than those presenting with atypical symptoms. The patients in the two groups did not differ significantly in sex, weight and height Z scores except age. Diarrhea (96.3%), abdominal distention (65.4%) and failure to thrive (60%) were the most common clinical presentations in the typical group, and short stature (62.5%) and anemia (31.2%) were the most common in the atypical group. Total/subtotal villous atrophy was significantly higher in the typical group than in the atypical group.ConclusionsMany children with celiac disease show an atypical form. The understanding of presentations of celiac disease may prevent delayed diagnosis. Celiac disease should be specially investigated in patients with recurrent iron deficiency anemia, short stature and autoimmune disorders.

Effects of cisapride on ventricular repolarization in children.

Life-threatening ventricular dysrhythmias mainly attributed to QTc prolongation have been reported in adults and children who were using cisapride, a prokinetic agent that facilitates gastrointestinal motility. Recent adult and paediatric case reports have suggested an association of malignant ventricular dysrhythmias with administration of cisapride in conjunction with drugs that inhibit its cytochrome P-450 metabolism. Therefore, to analyse the time- and dose-related effects of cisapride on ventricular repolarization, we prospectively studied infants and children receiving cisapride with no concomitant medications. Standard 12-lead resting ECGs were obtained from 38 patients (mean age: 6.6 +/- 4.4 y) before the first dose of cisapride (0.8-1.2 mg/kg/d) therapy, and 3 d, 7 d and 1 mo after the first dose of continuing cisapride therapy. The corrected QT interval (QTc), dispersion of QT and QTc (QTD, QTcD) were calculated. Patients were divided into two groups according to dose of cisapride: Group 1 (n = 22) (0.8 mg/kg/d), Group 2 (n = 16) (1.2 mg/ kg/d). Data obtained from these patients were compared with a control group consisting of 372 normal children. No clinical adverse effects such as palpitations, presyncope or syncope were noted during the study. Baseline QTc, QTD and QTcD measurements of the study group were not different from those of the control group. Mean QTc values of the study group on days 7 and 30 of cisapride therapy were found to be significantly higher than those of the control group (p < 0.001 and <0.0001, respectively). Mean QTc values of the study group on days 7 and 30 of therapy were also significantly higher than those of baseline value (p < 0.01 and <0.001, respectively). Mean QTD and mean QTcD values that were recorded throughout the cisapride treatment in the study group were not found to be different from the baseline values and the values of the controls. Mean QTD and QTcD were also not found to be different between Groups 1 and 2. However, mean QTc was found to be more significantly increased from baseline at the first month of therapy in Group 2 (p < 0.05). The results of this study suggest that cisapride treatment cause prolongation of ventricular repolarization without causing increased heterogeneity of repolarization (QT dispersion). However, the clinical significance of this effect is unclear, because all the patients in this study group remained asymptomatic, without signs of dysrhythmia.

Bilateral reexpansion pulmonary edema associated with pleural empyema: a case report

Reexpansion pulmonary edema is an uncommon complication following rapid reexpansion of the lungs. The risk increases with a prolonged duration of pulmonary collapse, the amount of drained liquid or air, and with decreased time of draining. Treatment is supportive. In general, the prognosis is favorable. A nine-year-old boy was presented with fever, cough, and respiratory distress. Pneumonia and left-sided pleural empyema were determined and a chest tube was emplaced. Clinical deterioration occurred in just a few minutes following chest tube insertion. His chest radiography revealed a pulmonary edema in the left lung. Despite mechanical ventilation, antibiotics, and diuretic treatment, no significant improvement occurred. Acute respiratory distress syndrome and multiple organ dysfunctions developed in the follow-up. The patient died on day 5 of hospitalization. In this report, a complicated reexpansion pulmonary edema with a lathal outcome in a 9-year-old child is presented.