Evaluation of bone mineral density in Turkish children with severe haemophilia A: Ankara hospital experience.

This study was conducted to understand the pathogenetic mechanisms that are involved in the development of bone loss in children with severe haemophilia A (HA). Fourty-four children with severe HA and 40 age- and gender-matched healthy control subjects were enrolled in this study. Markers of bone remodelling and osteoclast regulation including serum bone specific alkaline phosphatase, parathormone, 25-hydroxy-vitamin D(3) (25HOvitD(3)), osteocalcin and calcitonin levels were studied. Bone mineral density (BMD) was also studied in all children. The measurement of markers of bone remodelling and osteoclast regulation suggested increased osteoclast-mediated resorption activity in children with severe HA. Although serum parathormone levels were significantly increased, serum 25HOvitD(3) and osteocalcin levels were significantly reduced. BMD was significantly reduced in severe haemophilics compared with healthy controls. There was also significant inverse correlation between BMD z-score and total joint scores, and insignificant inverse correlation between BMD z-score and single joint scores. There were also significant inverse correlation between 25HOvitD(3) and osteocalcin levels and total joint scores. Children with severe HA could have significantly reduced BMD, compared with gender- and age-matched healthy control subjects. Our results of the markers of bone remodelling and osteoclast regulation suggested that increased osteoclast-mediated resorption and decreased osteoblastic activity in children with severe HA. All children with severe HA should be routinely screened in terms of BMD.

The effectiveness of radioisotope synovectomy for chronic synovitis in Turkish paediatric haemophiliacs: Ankara experience.

Between January 2006 and December 2009, 37 radioisotope synovectomy (RS) in 18 severe haemophilic patients (factor 8<1%) have been performed at our centre. The distribution of joint injected was as follows: rhenium-186 [Re186], 19 joints (ankles, 8 and elbows, 11) and yttrium-90 [Y90] 18 joints (knees, 18). Their mean age was 12 years (range: 8-20 years). Mean follow-up period after procedure was 22.6 months. We preferred to use Y90 for all large joints and Re186 for small joints. Haemophilic patients with grade-II or III synovitis were selected for RS in our study. Radioisotope synovectomy was performed in eight ankles for seven patients, 11 elbows for seven patients and 18 knees for 13 patients. Mean bleeding counts before the procedure and after the procedure were as follows: Ankles, 3.43 vs. 0.62 (P=0.002); elbows, 3.12 vs. 0.55 (P=0.000); and 3.83 vs. 0.62 (P=0.011). No major complications requiring secondary treatments were observed. In summary, an early RS is the best way to halt the evolution of chronic haemophilia synovitis to devastating haemophilic arthropathy. Radioisotope synovectomy is very effective and safe in the treatment of chronic synovitis of children with haemophilia. Our data confirm the efficacy of RS for the treatment of chronic haemophilic synovitis, which has been previously published by many authors around the world. We highly recommend this procedure for developing countries to prevent joint disabilities. For a better and a healthier generation, RS has to be introduced in all the developing countries.

Effect of anaesthetic agents administered to the mothers on transcutaneous bilirubin levels in the neonates.

AIM: To investigate the effect of anaesthetic agents on transcutaneous bilirubin levels during the first 24 h in neonates delivered by caesarean section. METHODS: A total of 168 neonates delivered by caesarean section, during which sevoflurane was used for general anaesthesia (group A), bupivacaine for spinal anaesthesia (group B), levobupivacaine for epidural anaesthesia (group C) and 155 neonates delivered vaginally were included in the study. Transcutaneous bilirubin levels (TBLs) of infants were measured during the first 24 h and compared with each other. RESULTS: The TBLs in neonates delivered vaginally were higher than those delivered by caesarean section, but the difference was not significant. TBLs were higher in groups A and C than in group B (p = 0.034, p = 0.011 respectively). TBLs were higher in group C than in group A, but the difference was not significant (p > 0.05). When the groups were compared with vaginal delivery group, TBLs in groups A and C were found higher (p = 0.03, p = 0.022 respectively). CONCLUSION: The route of delivery had no effect on TBL. While bupivacaine was found to have no effect on neonatal bilirubin levels, levobupivacaine increased neonatal biluribin levels, but further studies are needed for definite results.

Recent findings on pertussis epidemiology in Turkey.

The World Health Organization (WHO) reports that pertussis remains one of the least well-controlled vaccine-preventable diseases. It is supposed that the incidence of reported pertussis among adolescents, adults, and young infants has increased over the past decade. The aim of this study is to evaluate recent epidemiological data on pertussis in Turkey by regions. Data on vaccination coverage and pertussis incidence between 1986 and 2005 obtained from the Expanded Programme on Immunization national surveillance database of the Ministry of Health of Turkey were analyzed. Age and geographical distribution of the reported cases between 2000 and 2005 were evaluated. It was found that third-dose vaccination coverage increased from 1986 (45%) to 2005 (90%). In 2005, pertussis incidence tended to decrease (0.38 per 100,000) compared to 1986 (2.03 per 100,000). Even though only up to 6.5% of the cases were > or =15 years of age until 2005, 16.9% of them were included in this age group in 2005. It was observed that vaccination coverage rates steadily increased and pertussis incidence decreased by years despite some regional differences. In Turkey, pertussis incidence appears to be reaching the WHO targets, except East Anatolia. It is possible that waning immunity is responsible for the change of the age distribution of pertussis cases. However, priority should be given to strengthening available vaccination efforts throughout the country. A booster dose of pertussis vaccine in adolescence might be required in the future.

Pulmonary parenchymal alveolar histological study in experimental tracheo-oesophageal malformations / Estudio histológico alveolar y parenquimal pulmonar en malformaciones tráqueo-esofágicas experimentales

OBJECTIVE: Children operated on for tracheo-oesophageal malformation (TOM) often suffer from postoperative respiratory system difficulties. There is little current literature about this subject. This study aimed to investigate the causes of these problems in rats with experimental TOM by evaluating the lung alveolar histology. SUBJECTS AND METHODS: Twenty Wistar albino rats were used for the experiment. Twelve rats with a sperm positive vaginal smear received 1.75 mg/kg intraperitoneal adriamycin on days six to nine of gestation. A sham group was infused with saline instead of adriamycin. A control group was not subjected to any additional procedure. Their fetuses were dissected under surgical microscope. After examining the trachea and oesophagus, the lungs were dissected and fixed in 10% formalin. The groups were compared with respect to alveolar flat cell (Type-1), capillary density and air space percentage in the samples obtained under light microscopy. Statistical evaluation was performed through Mann-Whitney-U tests and Pearson Chi-squared tests. RESULTS: Type-1 cell ratio and air space percentage were the highest for the control and sham groups. However, the group that received adriamycin and developed TOM had the lowest values. There were no statistically significant differences between the groups with respect to capillary density. CONCLUSION: In rats with experimentally produced TOM, the pulmonary parenchyma showed delayed maturation. This could be the cause of the frequently seen respiratory system pathologies in children suffering from TOM. Further studies should be done to elucidate this.

OBJETIVO: Los niños operados por malformaciones tráqueo-esofágicas (MTE) sufren a menudo de dificultades postoperatorias en el sistema respiratorio. En el presente, existe poca literatura sobre este problema. Este estudio va encaminado a investigar las causas de estos problemas en ratas con MTE experimentales, mediante la evaluación de la histología alveolar del pulmón. SUJETOS Y MÉTODOS: Veinte ratas albinas Wistar fueron usadas en el experimento. Doce ratas con frotis vaginal positivo de esperma recibieron 1.75 mg/kg de adriamicina intraperitoneal en los días seis al nueve de gestación. A un grupo de simulación (sham group) se le dio una solución salina en lugar de adriamicina. El grupo de control no fue sometido a ningún procedimiento adicional. Sus fetos fueron disecados bajo microscopio quirúrgico. Luego de examinar la tráquea y el esófago, los pulmones fueron disecados y puestos en formaldehído al 10%. Recurriendo a la microscopía lumínica, los grupos fueron comparados con respecto a células alveolares planas (tipo 1), densidad capilar y porcentaje de espacio de aire en las muestras obtenidas. La evaluación estadística se realizó mediante tests U de Mann-Whitney y tests de Chi-cuadrado Pearson. RESULTADOS: La proporción de células de tipo 1 y el porcentaje de espacio de aire, fueron los más altos para los grupos de simulación y control. Sin embargo, el grupo que recibió adriamicina y desarrolló MTE, tuvo los valores más bajos. Con respecto a la densidad capilar, no hubo diferencias estadísticamente significativas entre los grupos. CONCLUSIÓN: En ratas con malformación MTE producida experimentalmente, el parénquima pulmonar mostró retardo en su maduración. Esto podría ser la causa de las patologías del sistema respiratorio observadas con frecuencia en niños que padecen de MTE. Se requieren estudios ulteriores a fin de dilucidar esta cuestión.

Pulmonary parenchymal alveolar histological study in experimental tracheo-oesophageal malformations.

OBJECTIVE: Children operated on for tracheo-oesophageal malformation (TOM) often suffer from postoperative respiratory system difficulties. There is little current literature about this subject. This study aimed to investigate the causes of these problems in rats with experimental TOM by evaluating the lung alveolar histology. SUBJECTS AND METHODS: Twenty Wistar albino rats were used for the experiment. Twelve rats with a sperm positive vaginal smear received 1.75 mg/kg intraperitoneal adriamycin on days six to nine of gestation. A sham group was infused with saline instead of adriamycin. A control group was not subjected to any additional procedure. Their fetuses were dissected under surgical microscope. After examining the trachea and oesophagus, the lungs were dissected and fixed in 10% formalin. The groups were compared with respect to alveolar flat cell (Type-1), capillary density and air space percentage in the samples obtained under light microscopy. Statistical evaluation was performed through Mann-Whitney-U tests and Pearson Chi-squared tests. RESULTS: Type-1 cell ratio and air space percentage were the highest for the control and sham groups. However, the group that received adriamycin and developed TOM had the lowest values. There were no statistically significant differences between the groups with respect to capillary density. CONCLUSION: In rats with experimentally produced TOM, the pulmonary parenchyma showed delayed maturation. This could be the cause of the frequently seen respiratory system pathologies in children suffering from TOM. Further studies should be done to elucidate this.

Latex allergy risk assessment in children and adolescents with type I diabetes mellitus.

An increased frequency of allergic reactions to latex has been reported in specific populations with chronic latex exposure. However, relevance of latex allergy to children and adolescents with type I diabetes mellitus (DM1) has not been studied yet. The aim of the studty is to assess latex allergy risk in children and adolescents with DM1. Thirty-nine children with DM1 and 35 controls were enrolled. In a case-control study, we applied to all subjects a standard questionnaire, and specific Immunoglobulin E (IgE) concentrations for latex, common aeroallergens, and food-allergens were measured in serum samples. Latex exposure rates by means of medical procedures, operations, and latex glove usage were not different between DM1 and controls. Symptoms due to latex exposure were not determined in both groups. Three (7.7%) subjects in DM1 tested positive for latex-specific antibodies (LSIE), whereas no subject in controls. Diabetics that tested positive for latex-specific antibodies had the disease for three, 5 and 8 years. Nine (23.1%) of diabetics, and two (5.7%) of controls were atopic (p = 0.04). In our investigation, we found that children and adolescents with DM1 are not a risk group for latex allergy, and LSIE in children with DM1 was not accompanied by symptoms of latex allergy, or, presumably, increased risk of latex anaphylaxis.

Evaluation of lung pathology in an experimental model of tracheal foreign body.

AIM: This experimental study was undertaken to evaluate the pathological changes caused by foreign body aspiration (FBA) on the lungs. MATERIAL AND METHODS: The study was performed on 19 Wistar albino rats. The rats in the control group (n = 5) were not subjected to any procedures. The rats in the sham group (n = 5) underwent tracheostomy, and a feeding tube was inserted into each rat. In nine rats in Group 1 (the study group), small lead marbles were placed into their trachea in addition to the procedures applied in the sham group. Lung tomography was performed on all the rats. The lungs, heart, and trachea were removed en bloc under ketamine hydrochloride anesthesia and fixed in 10 % formalin solution at the end of one week. The lung tissues were examined for FB, perivascular and peribronchial inflammatory cell infiltration, intra-alveolar bleeding, and post-obstructive emphysema. RESULTS: Tomography revealed FB in 4 rats and pathological evaluation showed FB in 7 rats. All the rats in Group 1 had peribronchial inflammation, while 7 rats (77.8 %) had perivascular inflammation. The sham and control groups were collectively referred to as Group 2 because of statistically similar findings. Group 1 and Group 2 significantly differed with respect to the findings of perivascular and peribronchial inflammation, intra-alveolar hemorrhage, and emphysema (p < 0.05). In Group 1, inflammation was detected in the opposite lobe of the lungs, although there was no FB. CONCLUSION: The presence of inflammation in the opposite lobe of the lungs where no FB was placed and in even those rats that expectorated the FB suggests that FBA is not a local pathology but a general pathology inducing inflammation in the whole lung. Thus, urgent removal of a foreign body is vital.

Response to growth hormone with respect to pubertal status on increased dose in idiopathic growth hormone deficiency: an analysis of Turkish children in the KIGS database (Pfizer International Growth Study).

AIM: To compare the growth response to growth hormone (GH) treatment in patients with idiopathic GH deficiency (IGHD) who were prepubertal with the response of those who were pubertal at the onset of GH therapy on an increased GH dose. PATIENTS AND METHODS: Among the Turkish patients enrolled in the Pfizer International Growth Study (KIGS) database with the diagnosis of IGHD, the growth data over 2 years of GH therapy were analyzed longitudinally of 113 (79 M) prepubertal (Group 1) and 44 (33 M) pubertal (Group 2) patients. Pubertal signs were reported to be present initially or to have appeared within 6 months of GH therapy in Group 2. Mean +/- SD age at onset of therapy was 8.7 +/- 3.5 and 13.5 +/- 1.8 years; height SDS -4.2 +/- 1.4 and -3.2 +/- 1.1 (p < 0.05) in Groups 1 and 2, respectively. Mid-parental height (MPH) SDS did not show a significant difference between the two groups (-1.5 +/- 1.1 vs -1.7 +/- 1.1). RESULTS: Delta height SDS over 2 years of therapy was significantly higher in Group 1 (1.1 +/- 1.0) than in Group 2 (0.7 +/- 0.6) (p <0.05) in spite of a significantly lower dose of GH (14.6 +/- 3.3 in Group 1 vs 17.0 +/- 3.1 IU/m2/week in Group 2, p < 0.05). Ht--MPH SDS showed an increase from -2.4 +/- 1.7 to -1.4 +/- 1.5 in Group 1 and from -1.5 +/- 1.5 to -0.8 +/- 1.3 in Group 2. Overall delta height SDS showed negative correlations with age (r = -0.32), height SDS (r = -0.41) and height--MPH SDS (r = -0.40) at onset of therapy (p < 0.001). CONCLUSIONS: These data show that in IGHD the slight increase (15-20%) in the dose of GH during puberty was not adequate to maintain height velocity at the same magnitude as in prepuberty, and thus was not cost effective.

A non-accidental poisoning with ammonia in adolescence.

Abstract Introduction Ammonia is an important chemical agent used in industry. Accidental inhalation of ammonia has resulted in upper airway and bronchoalveolar injury. If a large amount of ammonia is breathed, the agent can cause unconsciousness, shock and even death. Case In this article, we report a case of non-accidental poisoning in adolescence. The patient was a 14-year-old boy and working as an apprentice at a workshop of a jeweller. He had drunk some amount of diluted ammonia by the force of another, 18-year-old, male apprentice. This patient presented with ammonia intoxication symptoms culminating in coma. Conclusions We reported this case as an unusual ammonia poisoning seen in adolescence.

A rare extrahepatic syndrome related to acute hepatitis type B: epididymitis in an adolescent.

Hepatitis B is a common, vaccine-preventable infection with high mortality and morbidity rates worldwide. Numerous extrahepatic syndromes have been described in patients with either acute or chronic viral hepatitis B. But there is no previous report of co-existence of HBV infection and epididymitis in the English literature. We would like to present and discuss a 12-year-old male patient with epididymitis that might be relation exist with an underlying acute hepatitis B infection.

Multiple dose IVIG treatment in neonatal immune hemolytic jaundice.

Isoimmune hemolytic jaundice due to ABO and Rh blood group incompatibility is an important problem in the neonatal period. Intravenous immune globulin (IVIG) treatment in isoimmune jaundice has been shown to be effective, but the response to treatment is variable. In this study, the effect of multiple doses IVIG therapy versus single dose MG therapy was investigated in 61 babies who had ABO and Rh hemolytic disease. Patients were divided into three groups. Group I received multiple dose IVIG treatment, group II received single dose MG treatment, and group III was not given any IVIG. All three groups received phototherapy. No exchange transfusion was needed in group I. The rate of exchange transfusion was 12 per cent in group II and 33 per cent in group III. Duration of phototherapy was shorter in group I than in groups II and III. It was concluded that IVIG treatment reduces the need of exchange transfusion in neonatal isoimmune hemolytic jaundice by lowering hemolysis. Multiple doses IVIG treatment appears to be better at blocking ongoing hemolysis.

Hajdu-Cheney syndrome with growth hormone deficiency and neuropathy.

A Hajdu-Cheney syndrome is a very rare congenital dysplastic bone disease including acro-osteolysis, short stature, characteristic facies, osteopenia, abnormalities of spine, skull and long bones. A 9 year-old boy presented at our clinic with a chief complaint of short stature and frequent lower respiratory tract infections. He had typical physical and radiographic features of Hajdu-Cheney syndrome associated with growth hormone (GH) deficiency and peripheral motor neuropathy. To our knowledge, this is the first report describing GH deficiency and neuropathy in Hajdu-Cheney syndrome.

Heights and weights of primary school children of different social background in Ankara, Turkey.

A cross-sectional anthropometric survey was carried out in a low socio-economic and high socio-economic region of Ankara, Turkey, to measure the weights and heights of school children. The study group consisted of 5289 children between the ages of 5 and 11 years. Both boys and girls from the high socio-economic group had superior body measurements compared to those of the low socio-economic group. The difference between the mean weight for age values of two groups was statistically significant (P < 0.05), whereas no statistically significant difference was found on the basis of height for age values among all age groups. To make a comparison both with National Centre for Health Statistics and World Health Organisation (NCHS-WHO) standards and Turkish standards we used the data from high socio-economic group only. Our results showed that the mean height and weight values of boys and girls were higher than the 50th centile height and weight values of NCHS-WHO standards. Almost 25 years have passed since the measurements of Turkish standards were taken. The height differences were in the range of 0.24-1.51 cm/decade, with a mean value of 0.96 cm/ decade. These results led us to conclude that, local/regional standards for height and weight are needed, and repeated assessments are useful for follow-up of populations.