Evaluation of Selenium Levels and Mean Platelet Volume in Patients with Simple Febrile Convulsion

Objective: This study aimed to evaluate serum selenium levels and mean platelet volume in children who experience simple febrile convulsion. Methods: The study comprised 42 patients diagnosed with simple febrile convulsions and a control group of 30 healthy children. Blood samples were taken following a febrile convulsion. Selenium levels in the serum of both the patients and control subjects were measured with the hydride formation method on an atomic absorption spectrometry device and mean platelet volume was evaluated. Findings: When the mean values of the febrile convulsion patients were compared with those of the control group, the mean selenium levels and thrombocyte count were found to be statistically significantly low (P=0.002, P=0.01 respectively) and the mean platelet volume values were statistically significantly high (P=0.002). Conclusion: While low serum selenium levels cause the onset of a febrile seizure in patients with simple febrile convulsion, it is thought that the increased mean platelet volume shows infection activity causing febrile convulsion.Key Words: Febrile Convulsion, Selenium, Platelet: Mean Platelet Volume, Antioxidant     

Zinc and Copper Status in Children with Beta-Thalassemia Major

Objective

There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia major.

Methods

In this cross sectional study all children under 12 years affected by beta thalassemia major (40 patients) were evaluated for serum zinc and copper levels in Qazvin thalassemia center (Qazvin, Iran) in 2007. Serum measurements for zinc and copper were performed by atomic absorption spectrophotometer.

Findings

The mean concentrations of serum zinc and copper levels were 67.35±20.38 and 152.42±24.17 µg/dl respectively. Twenty-six (65%) of thalassemic patients had zinc concentration under 70 µg/dl (hypozincemia). None of the thalassemic children had copper deficiency. No significant correlation between serum zinc level with age, weight, height, body mass index, duration of blood transfusion, desferrioxamine dose and ferritin level was observed in thalassemic patients (P=0.3).

Conclusion

This study revealed that hypozincemia is common in thalassemic patients, but in contrast, there is no copper deficiency. Further evaluation in this regard is recommended.     

Quality of Life (QoL) and the Factors Affecting it in Transfusion-dependent Thalassemic Children

Objectives

To evaluate the Quality of life (QoL) and the factors affecting it in transfusion-dependent thalassemic children.

Methods

Current study was conducted at Department of Pediatrics, Dr. SNMC, Jodhpur. Ninety-three transfusion-dependent thalassemic children aged between 8 and 18 y were enrolled. Quality of life was assessed using Pediatric Quality of Life (PedsQL) measurement model in thalassemic children.

Results

Mean age of children was 11.8?±?3.2 y. Lowest mean score was observed in School functioning (57.4) while in Physical functioning, Emotional functioning and Social functioning it was 73.9, 82.2 and 83.49 respectively. Mean PedsQL score was higher in 8-12 y age group as compared to 13-18 y (p?=?0.024). PedsQL score was higher in children who required transfusion once in a month (p?=?0.028) and children on combined chelation therapy as compared to children who were on injectable chelator (p?=?0.039). Health Related QoL was not affected with gender, education status, family history of thalassemia, ferritin level, splenectomy or consanguinity.

Conclusions

Factors related with low QoL in current study were higher age, increased transfusion frequency and injectable mode of chelation. In order to improve the QoL in thalassemic children appropriate programmes and interventions should be started targeting above domains.

     

Hypoglykämien mit neurologischen Komplikationen bei Kindern mit Typ-1-Diabetes im Vorschulalter

Rationale. Preschool children with type 1 diabetes have a high incidence of severe hypoglycemia with convulsions or loss of consciousness. Prevention of severe hypoglycemias is a preeminent goal in the long-term care of diabetic children. Methods. Twenty-four preschool children with diabetes (age ≤6 yrs) were prospectively studied for 12 months. Incidence of severe hypoglycemic episodes and neurologic symptoms were registered and related to diabetes control and therapy. Severe hypoglycemia was defined as blood glucose below 60 mg/dl and severe neurologic signs (convulsions, loss of consciousness, paresis). Results. Five episodes of severe hypoglycemia occurred in 5 out of 24 preschool children (incidence 0.21/yr). Major signs were generalized convulsions (2 patients), focal convulsion without loss of consciousness (2 patients) and isolated transient hemiparesis in 1 patient. All episodes occurred at night between 11.30 p. m. and 4.20 a. m.. Causes identified in retrospect were an inadequately high basal insulin dose at bedtime in 4 children and an insufficient reduction of insulin after increased physical activity in one child. In the 5 children with severe hypoglycemia BMI was significantly (p = 0.015) higher in the 5 children with severe hypoglycemia compared with those without severe hypoglycemias. Age, duration of diabetes, average HbA1c levels, daily insulin dose, number of insulin injections and number of blood glucose tests were not different. Conclusions. All episodes of severe hypoglycemias in preschool children occurred at night between 11.30 p. m. and 4.20 a. m. The most likely cause was too much basal insulin at bedtime. Families with young diabetic children should be alert to the danger of high insulin doses at bedtime and the necessity of early dose reductions. A high body mass index was associated with the occurrence of severe hypoglycemia, while a low HbA1c value by itself was not.     

Deferoxamine-induced dysplasia-like skeletal abnormalities at radiography and MRI

Background

Current thalassemia major treatment includes blood transfusion and iron chelation, which is associated with growth disturbances and radiographic changes in the long bone metaphyses.

Objective

To explore and discuss the spectrum of deferoxamine-induced bone-dysplasia-like changes in children with thalassemia major in Egypt.

Materials and methods

We studied 59 Egyptian children with thalassemia major and generalized arthralgia. All started deferoxamine treatment at 3 years of age. We conducted skeletal survey and MRI of both knees in radiographically positive children. Each child’s age, serum ferritin, age of onset and duration of therapy were compared with the radiologic findings.

Results

Twenty-two (37.3%) children had variable degrees of skeletal dysplasia-like changes similar to those described with deferoxamine intake, mostly around the knees. Mild dysplasia-like changes were seen in 4 (18%) children; moderate changes were seen in 11 (50%) children and severe changes were seen in 7 (31.8%) children. No statistically significant relationships were detected between bone changes and the children’s age, age of starting deferoxamine, duration of therapy, or serum ferritin level.

Conclusion

A wider spectrum of deferoxamine-induced bone-dysplasia-like changes was recognized despite delayed onset and small doses of therapy. These changes should be considered as a possible cause of arthropathy in children with thalassemia major, especially symptomatic children.     

Dietary habits and nutritional status of children from Ukraine during the first 3 years of life

Aim: The aim of our study was to assess the nutritional status and eating behavior of young children and identify the prevalence of macro- and micronutrient diet deficiencies in Ukraine. Materials and methods: Three hundred and fifty children aged from 9 months to 3 years from central, eastern and western regions of Ukraine were involved in the cross-sectional study. Basic child's data were collected, health status was assessed by a physician, and parents used a 3-day food diary and a food questionnaire for self-completion. Data from the diaries and questionnaires were analyzed with DietPlan 6 software. Results: The diet composition was mostly adequate for age. Overall average provision with energy (1165.67 [29.67–4951.33] kcal/day), protein (40.53 [0.63–230.37] g/day) and carbohydrates (153.63 [3.53–708.7] g/day) exceeded the corresponding standards. The diet of the majority of children did not comply with the recommended intake of zinc (91%), iron (68%), calcium (61%), iodine (49%), vitamins A (99%), D (97%), B₆ (89%), B₁₂ (71%), E (70%) and B₁ (61%). Excessive weight was significantly associated with higher levels of energy, protein, carbohydrates and fat consumption. Overweight was reliably correlated with a higher diet's energy and carbohydrates content. Conclusions: The contemporary diet of young children in Ukraine, like in many other developed countries, is generally unbalanced, containing an excess of energy and protein as well as inadequate amount of many minerals and vitamins.     

Fat-Soluble Antioxidant Vitamins,Iron Overload and Chronic Malnutrition in β-Thalassemia Major

Objective

To assess the antioxidant vitamins A (retinol) and E (α-tocopherol) levels, iron status and growth retardation in children with β-thalassemia major in Odisha, an eastern state of India.

Methods

Forty three children aged 1–15 y diagnosed with β-thalassemia major (28 males and 15 females) and 42 age-matched healthy controls (22 males and 20 females) were studied. β-thalassemia was detected by using Bio-rad variant assay. Measurement of blood hemoglobin (Hb), hematocrit, serum vitamins (A and E) and ferritin was carried out by standard methods.

Results

Mean hemoglobin (6.60?±?1.84 vs. 11.8?±?2.29 g/dL, p?<?0.01), serum retinol (28.0?±?17.67 vs. 54.4?±?36.56 μg/dL, p?<?0.001) and α-tocopherol (0.2?±?0.34 vs. 1.1?±?0.82 mg/dL, p?<?0.001) were significantly lower in children with thalassemia compared with control group, however, serum ferritin (storage iron) was elevated in thalassemia patients (553.7?±?176.80 vs. 57.3?±?40.73 ng/mL, p?<?0.001). Vitamin E had significantly correlated with hemoglobin and hematocrit values in the patients. Growth retardation in terms of stunting (79 % vs. 24 %, p?<?0.0001) and thinness (32.6 % vs. 9.5 %, p?<?0.05) was significantly higher in thalassemic children compared with normal children.

Conclusions

This study shows that children with β-thalas-semia major are in a state of oxidative stress of hyperfer-ritinemia with deprived antioxidant vitamins (A and E) and poor growth status suggesting a possible need for reduction in iron overload and additional antioxidant supplementation.     

Störungen der Glukosetoleranz bei Thalassaemia major

Background. In patients suffering from thalassemia major, hemosiderosis frequently causes endocrine disorders. We investigated the development of glucose tolerance disorders and the influence of therapeutical interventions such as intensified chelation therapy and diet. Methods and patients. 60 patients with thalassemia major, ages ranging from 4 to 36 years, treated regularly with both subcutaneous desferrioxamine infusions and erythrocyte transfusions, were investigated for endocrine disorders. Oral and intravenous glucose tolerance tests as well as intravenous glucagon challenge were used to investigate the insulin secretion. In patients with impaired glucose tolerance the influence of intensified (intravenous) chelation therapy and dietary interventions on blood glucose and serum insulin levels were studied. Results. In patients with thalassemia major, disturbed glucose tolerance appears to be one of the four most common endocrine disorders in the second decade of life and later. In the early stages of the disease, glucose tolerance disorders were associated with hyperinsulinemia; in later stages insulinopenic diabetes develops due to β-cell exhaustion. In some patients in the early stages of the disease, intensified chelation therapy or dietary treatment only improved their glucose tolerance. Conclusion. Especially for thalassemia patients in the early stages of glucose tolerance disorders, intensified desferrioxamine treatment and diet can be of benefit and in some cases retard the manifestation of diabetes mellitus. These treatments do not, however, change the requirement for insulin substitution at an advanced stage of disturbed glucose tolerance.     

Quality Matters–Hematopoietic Stem Cell Transplantation <Emphasis Type="Italic">versus</Emphasis> Transfusion and Chelation in Thalassemia Major

Objective

To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion.

Methods

The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale.

Results

The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92.8, respectively in transplant group and 83, 82.7 and 83.6, respectively in children in transfusion group. The adult group on transfusion showed overall poorer scores of 74.9, 76 and 73.9, respectively. The average scores in all domains were significantly (P<0.05) lower and drop steeply in second decade in transfusion group.

Conclusion

Allogeneic stem cell transplantation improves quality of life in thalassemia major.

     

Psychological Aspects in Children and Adolescents With Major Thalassemia: A Case-Control Study

Background:

Thalassemia is an inherited blood disease. It is a serious public health problem throughout the Mediterranean region, the Middle East and the Indian subcontinent, as well as in Southeast Asia.

Objectives:

Thalassemia is an inherited blood disease. It is a serious public health problem. In this study we assessed psychological aspects in Iranian children and adolescents with thalassemia major.

Patients and Methods:

In this case-control study sixty healthy subjects aged 7-18 years and Sixty Patients with confirmed diagnosis of major thalassemia were enrolled. After obtaining informed consent from parents of all participating thalassemia patients and healthycontrols, we assessed psychological aspects and quality of life by Pediatric Quality of LifeTM (PedsQL™), Strengths and Difficulties Questionnaires (SDQ), State and Trait Anxiety, Children''s Depression Inventory (CDI).

Results:

The results of this study indicate that there are significant changes in depression, anxiety, QOL and behavioral screening between children with thalassemia major compared with healthy subjects by means of both parents and children reports. According to the results, children with thalassemia major have more psychological problems than healthy ones. Patients with thalassemia have a lower QOL than their peers (P = 0.001), the rate of depression is higher in this group (P = 0.015), Also behavioral problems in these children are more than healthy subjects (P = 0.009).

Conclusions:

We recommend appropriate treatment and counseling procedures in addition to specific treatment of thalassemia. According to the results we suggest to establish pediatric psychiatric clinics beside thalassemic clinics to cure psychological aspects of the disease.     

Serum sodium levels and probability of recurrent febrile convulsions

In a prospective study of 69 children with febrile convulsions, serum sodium levels were often lower than normal (52% had levels <135 mmol/l). The mean level (134.4±0.4 mmol/l) was significantly lower as compared to a group of children without fever (140.6±0.4 mmol/l,n=23) and as compared to a group with fever but without convulsions (137.6±0.6 mmol/l,n=31). The probability of a repeat convulsion within the same febrile period appeared to be significantly related to the serum sodium level.Conclusion Measurement of the serum sodium is a valuable investigation in the child with a febrile convulsion. The lower the serum sodium level, the higher the probability of a repeat convulsion. This knowledge may be of practical value in deciding whether to admit the child or allow it to return home and in advising parents or carers of the risk of a repeat convulsion.     

88例热性惊厥患儿缺铁性贫血相关指标分析

目的了解热性惊厥与缺铁性贫血的关系。方法检测88例热性惊厥患儿的红细胞计数、血红蛋白、红细胞平均容积、红细胞平均血红蛋白、红细胞平均血红蛋白浓度、血清铁、血清铁蛋白,并以同期住院的76例呼吸道、肠道感染而无惊厥患儿为对照组,将两组数据进行统计分析。结果热性惊厥组缺铁性贫血的发生率为61.36%,对照组为43.42%,血红蛋白、血清铁含量与对照组有显著性差异(P<0.05);而且复杂型热性惊厥的缺铁性贫血的发生率占85%,与单纯型比较亦有显著性差异(P<0.05)。结论血清铁与小儿热性惊厥密切相关,缺铁性贫血可能是引起热性惊厥的原因之一。     

Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major

Objectives

To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.

Methods

A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit.

Results

β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among β-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio.

Conclusions

Serum hepcidin is elevated in children with β-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.

     

Kardiomiopatia restrykcyjna – historia ze szczęśliwym zakończeniem

Cardiomyopathies are a group of rare diseases of a myocardium in a pediatric population which affect 1–1.5 cases per 100 000 children. Restrictive cardiomyopathy (RCM) is the least common type, which constitutes 2–5% of all cardiomyopathies. RCM is characterized by a diastolic dysfunction which is a result of an increased stiffness of the ventricular myocardium with a normal systolic function. Differential diagnosis should include especially constrictive pericarditis. Because of a poor prognosis, it is important to diagnose patients as soon as possible and to early qualify them for a heart transplantation.     

Correlation between umbilical cord blood lipid profile and neonatal birth weight

Introduction and aim: The aim of this study was to investigate the relation between birth weight and neonatal umbilical cord serum lipid levels.     

MRI for Quantification of Liver and Cardiac Iron in Thalassemia Major Patients: Pilot Study in Indian Population

Objective

To evaluate Magnetic resonance imaging (MRI) as a tool to quantify liver and cardiac iron in Indian population with thalassemia major, and correlate liver and cardiac iron values with that of serum ferritin (SF).

Methods

Fifty patients aged between 8 to 18 y, with thalassemia major on regular blood transfusions and oral iron chelation therapy were enroled in the study. Twenty patients within the same age group, having no history of blood transfusions and no liver or cardiac disease were taken as controls. T2* MRI of heart and liver and SF estimation was done for all the cases as well as controls. All MRI scans were done on a 1.5-T Siemens MRI scanner using body coil.

Results

The mean SF among cases was 2150 ng/ml (SD 2179). Significant correlation was found in patients between liver iron concentration (LIC, mean 15) and SF levels (r = 0.522; p < 0.001), and also significant but weaker correlation was found in patients between myocardial iron concentration (MIC, mean 1.3) and SF levels (r = 0.483; p < 0.001). Seventeen (34%) patients had a SF of <1000 ng/ml. Of these, 11 and 3 patients respectively had LIC and MIC more than normal range.

Conclusions

T2* MRI is a valuable non-invasive tool for quantification of liver and cardiac iron deposition in patients with thalassemia major. It can demonstrate high LIC and MIC, even though the targeted SF levels are low in thalassemia, indicating the need for escalation of the chelation therapy. This needs to be confirmed on full-fledged larger prospective studies.

     

Clinical characteristics of febrile convulsions during primary HHV-6 infection

OBJECTIVE—To clarify clinical characteristics of children with febrile convulsions during primary human herpesvirus 6 (HHV-6) infection.SUBJECTS AND METHODS—The clinical characteristics of first febrile convulsion were compared between those with and without primary HHV-6 infection in 105 children. HHV-6 infection was verified by culture or acute/convalescent anti-HHV-6 antibody titres.RESULTS—Primary infection with HHV-6 was seen in 21 of 105 patients with febrile convulsions (3 upper respiratory infection, 1 lower respiratory infection, and 17 exanthem subitum). 13 of 23 patients < 1 year, 19 of 79 patients with first febrile convulsion, and 2 of 15 with second convulsion were infected with HHV-6. The median age of patients with first febrile convulsion and HHV-6 was significantly lower than those without infection. The frequency of clustering seizures, long lasting seizures, partial seizures, and postictal paralysis was significantly higher among those with primary HHV-6 infection than among those without. The frequency of atypical seizures in 19 patients with first febrile convulsion associated with primary infection was significantly higher than in 60 patients without primary infection. The frequency in infants younger than 1 year of age was also significantly higher than that in 10 age matched infants without primary infection.CONCLUSIONS—These findings suggest that primary infection with HHV-6 is frequently associated with febrile convulsions in infants and young children and that it often results in the development of a more severe form of convulsions, such as partial seizures, prolonged seizures, and repeated seizures, and might be a risk factor for subsequent development of epilepsy.     

Hypozincaemia in febrile convulsion

To understand further the role of trace elements in the pathogenesis of febrile convulsions, serum zinc (Zn), copper (Cu), magnesium (Mg) and CSF Zn, Cu, Mg and protein levels were measured by spectrometry in patients with febrile convulsion (n=19), bacterial meningitis (n=9), viral CNS infection (n=16) and in the control groupn=10) which consisted of children with signs of meningeal irritation due to upper respiratory tract infection but normal CSF findings. Samples were obtained within 6 h after admission to hospital. Mean serum and CSF Zn levels in the febrile convulsion group were significantly lower than in the other groups (for serum Zn: 0.66±0.03 mg/l vs 0.98±0.07 mg/l, 1.06±0.08 mg/l, 1.05±0.09 mg/lP<0.05; for CSF Zn: 22.96±1.62 g/l vs 75.47 ±6.9 g/l, 50.32±5.235 g/l, 39.85 ±2.81 g/lP<0.05). A linear relationship was established between serum Zn and CSF Zn levels (P<0.001). Mean CSF Zn, Cu and protein levels in the bacterial meningitis group were significantly higher than in the other groups (for CSF Cu 63.94±6.33 g/l vs 38.77±2.70 g/l, 35.84±3.48 g/l, 33.86±2.88 g/lP<0.05; for CSF protein 0.80 ± 0.12 g/l vs 0.22±0.02 g/l, 0.53±0.08 g/l, 0.19±0.01 g/lP<0.05). In children with meningitis, the elevation of the mean CSF Zn and Cu levels may result from the breakdown of the blood-brain barrier and subsequent leakage of trace elements and protein from serum to CSF. There was no significant difference between the four groups in terms of mean serum Mg and mean CSF Mg levels.Conclusion Serum and CSF Zn levels are decreased in children with febrile seizures. Zinc deprivation may play a role in the pathogenesis of febrile seizures.     

Clinical characteristics of febrile convulsions during primary HHV-6 infection.

OBJECTIVE: To clarify clinical characteristics of children with febrile convulsions during primary human herpesvirus 6 (HHV-6) infection. SUBJECTS AND METHODS: The clinical characteristics of first febrile convulsion were compared between those with and without primary HHV-6 infection in 105 children. HHV-6 infection was verified by culture or acute/convalescent anti-HHV-6 antibody titres. RESULTS: Primary infection with HHV-6 was seen in 21 of 105 patients with febrile convulsions (3 upper respiratory infection, 1 lower respiratory infection, and 17 exanthem subitum). 13 of 23 patients < 1 year, 19 of 79 patients with first febrile convulsion, and 2 of 15 with second convulsion were infected with HHV-6. The median age of patients with first febrile convulsion and HHV-6 was significantly lower than those without infection. The frequency of clustering seizures, long lasting seizures, partial seizures, and postictal paralysis was significantly higher among those with primary HHV-6 infection than among those without. The frequency of atypical seizures in 19 patients with first febrile convulsion associated with primary infection was significantly higher than in 60 patients without primary infection. The frequency in infants younger than 1 year of age was also significantly higher than that in 10 age matched infants without primary infection. CONCLUSIONS: These findings suggest that primary infection with HHV-6 is frequently associated with febrile convulsions in infants and young children and that it often results in the development of a more severe form of convulsions, such as partial seizures, prolonged seizures, and repeated seizures, and might be a risk factor for subsequent development of epilepsy.     

Bestimmung von Insulinresistenz und Insulinsensitivität bei Kindern und Jugendlichen

Background. The hyperinsulinimic euglycemic clamp is the gold standard to determine insulin action. In adults simpler methods to define insulin resistance (HOMA) and -sensitivity (ISI) are available. In children/adolescents HOMA and ISI were evaluated and correlated with BMI and blood pressure. Patients. 59 children/adolescents of comparable age were divided into a control group: 23 patients, the Ullrich-Turner group: 14 girls, the obese group: 22 patients. Results. HOMA increased from controls to obesity, while ISI decreased. The association between HOMA and ISI was hyperbolic with R=0.88. The correlation between HOMA and BMI and blood presure was positive and negative for ISI. In one patient the coefficient of variation for HOMA was 30% and 26% for ISI. Conclusion. It appears that in children and adolescents HOMA and ISI may be suitable methods to determine insulin action.