Non-Cardiac Cause of Death in Selected Group Children with Cardiac Pathology: A Retrospective Single Institute Study.

BACKGROUND: Pediatricians and pediatric surgeons often face children with cardiomegaly and dilatative or hypertrophic cardiomyopathies presenting with or without symptoms. Some of these patients have already been diagnosed and received medication, and some present with completely unrelated pathologies. METHODS: We performed a 4-year retrospective study on the causes and mechanisms of death of children with cardiac pathology who died outside the cardiology clinic of our hospital by studying the hospital charts and necropsy reports. All children who were in this situation in our hospital were included. RESULTS: Most children in our study group were infants (81.82%), most were boys (81.82%), and in most cases, the cause or mechanism of death was unrelated to their heart condition, whether it had already been diagnosed or not (one case probably died as a result of a malignant ventricular arrhythmia). Additionally, 27.27% of children died as a consequence of bronchopneumonia, the same percentage died as a consequence of an acquired non-pulmonary disease or after surgery, and 18.18% died as a consequence of congenital malformations. CONCLUSIONS: Cardiac disease needs to be thoroughly investigated using multiple tools for all children presenting with heart failure symptoms, those with heart murmurs, and children scheduled for surgery of any type. The intensive care specialist and surgeon need to be aware of any heart pathology before non-cardiac surgical interventions.

[Allergic comorbidities in bronchial asthma, in children]. / Comorbiditati alergice în astmul bronsic la copil.

Bronchial asthma is the most frequent chronic disease of childhood. The association of this disease with allergic rhinitis increases the prevalence and severity of bronchial asthma to 60% compared with 2% in subjects without allergic rhinitis. Food allergy can be expressed in a variety of respiratory symptoms, especially when IgE mediated immune responses are involved. In children, the prevalence of bronchial asthma associated with food induced atopic eczema is 2-8% and respiratory clinical picture can be a component of the multisystem anaphylactic reaction or of chronic respiratory disease like bronchial asthma, serous otitis. The association of other allergic comorbidities, like sinusitis, ocular allergies, lymphoid hypertrophy, sleep obstructive apnoea, leads to the conclusion that allergic bronchial asthma (extrinsic) is a systemic disease, with onset at any age, and the dominant clinical feature depends on the child's genetic pattern.

[Chronic hepatitis B--still a problem in children]. / Hepatita cronica virala B--o problema încâ în actualitate la copil.

Vaccination of the newborns and adolescents had impact on the incidence of acute and chronic infection with hepatitis B virus (HBV) in children. After a decade of this national program application, authors proposed to study the epidemiologic dynamics of chronic infection with HBV in this interval, compared with the previous period, prior to vaccination.

[Osteopenia in children with juvenile idiopathic arthritis]. / Osteopenia la copiii cu artrita juvenila idiopatica.

UNLABELLED: The aim of the study was to evaluate the presence and etiopathogenesis of osteopenia in 41 children with Juvenile Idiopathic Arthritis (JIA). METHODS: Bone status was evaluated by quantitative ultrasound using a Sunlight Omnisense 7000s Ultrasound Bone Sonometer. Measurements were performed at the distal radius and midshaft tibia. Results were obtained as Speed of sound (SOS) and Z-score. We used standardised clinical evaluation (modified Giannini's criteria, CHAQ). ESR, Fibrinogen, serum calcium, magnesium, alkaline phosphatase, protein electrophoresis, 25-OH vitamin D (RIA) and urinary Hydroxyproline were obtained in all patients. Osteopenia was present in 15 (36.5%) patients. Statistical analysis was performed with SPSS 13.0. RESULTS: Age, sex, age at onset, disease duration, life standards and duration of corticotherapy and methotrexate treatment were not related to osteopenia in our study. The disease activity, evaluated by clinical criteria, ESR and Fibrinogen, was strongly associated with osteopenia (p<0.001). Nutritional status was an independent risk factor for osteopenia (p<0.001). Low serum calcium (p=0.034), magnesium (p=0.010), 25-OH vitamin D (p=0.091) and alkaline phosphatase (p=0.31) were more frequent in patients with osteopenia. Hydroxyproline was increased in all patients with osteopenia (p<0.001). CONCLUSIONS: Osteopenia was a frequent (36.5%) complication of JIA in our study. The disease activity and nutritional status were the most important risk factors for osteopenia. The increase of bone reabsorption was the main pathogenic mechanism of osteopenia in our study. Calcium and magnesium deficits were related to osteopenia. Decrease of bone synthesis was not associated with osteopenia in the present study.

Glycogen storage disease type I--between chronic ambulatory follow-up and pediatric emergency.

BACKGROUND AND AIMS: To describe the characteristics of patients with type I glycogenosis, the presentation types, the main clinical signs, the diagnostic criteria and also the disease outcomes on long term follow-up. METHODS: The study group consisted of 6 patients (medium age 3 years 6 months) admitted in hospital between 2001 and 2005 and followed-up for 1 to 5 years. The sex ratio was 1:1. RESULTS: The referral reasons varied from hepatomegaly incidentally discovered (3 of 6 patients) to abdominal pain (4 of 6 patients), growth failure (3 of 6 patients), symptoms of hypoglycemia (3 of 6 patients), recurrent epistaxis (1 patient). Hepatomegaly was present in all cases. Biological profile: hypoglycemia, increased transaminase values, hypertriglyceridemia, lactic acidosis, normal uric acid levels. Two patients had neutropenia and other two had increased glomerular filtration rate. Liver biopsy showed glycogen-laden hepatocytes and markedly increased fat. Four patients had type Ia and 2 patients type Ib glycogenosis. The therapy consisted of: diet, ursodeoxycholic acid, granulocyte colony-stimulating factor, broad spectrum antibiotics for those with type Ib glycogenosis. The follow-up parameters were clinical, biological, imaging. Metabolic interventions and antiinfectious therapy were necessary. All patients are alive, two of them on the waiting list for liver transplantation. CONCLUSIONS: Glycogen storage disease type I is a rare condition, but with possible life-threatening consequences. It has to be kept in mind whenever important hepatomegaly and/or hypoglycemia are present.

[Obesity in children: new controversies and present prevention measures]. / Obezitatea copilului--noi paradigme si masuri preventive actuale.

The authors realise a synthesis on classical data and recent pathogenic explanations in childhood obesity. The obesity is a nutritional disorder of great interest nowadays and surpasses the incidence of the major problem of pediatrics until now--the malnutrition. There is documented data concerning the global incidence of obesity which is continuously growing when it comes to children. That is why the prophylaxis must become a priority by using measures in the first period of life (natural feeding, the need of late diversification, the avoidance of hyperproteic diets). The recent pathogenic data and the long term populational studies change the old conceptions regarding the risk of some categories of children. Thus mother's malnutrition, the low birth weight, children that followed hyperproteic diets paradoxically represent categories of risk for obesity. A recent recorded phenomenon, which amplifies the risk for obesity is the early adiposity rebound which is recorded nowadays even for ages lower than five years. There are described the hormonal mechanisms involved in appetite and satiety up to the receptor level: leptin, ghrelin, adiponectin, endocannabinoid receptors. There are pointed out all the long term risk elements (high birth weight, low birth weight, the pregnant woman's nutrition) and the modern medical treatments for obesity.

[The effects of inhaled corticoids on bone density in asthmatic children]. / Efectele corticoterapiei inhalatorii asupra densitatii osoase la copiii cu astm bronsic.

OBJECTIVE: To evaluate bone status of asthmatic children on chronic inhaled corticoid therapy. METHODS: Bone densitometry was performed by Quantitative ultrasound (QUS) of the distal radius in 74 asthmatic children, evaluating Z-score; serum levels of 25 hydroxyvitamin D were measured in 10 cases. RESULTS: 28 of 74 children had osteopenia, defined as Z scores lower than -1. Statistic analyzes showed a significant correlation (p <0.05) of osteopenia with the duration of inhaled corticoid therapy (CSI), the absence of correlation with the dose of CSI, age, sex, severity step and duration of the disease. 25 hydroxyvitamin D was low in 8 of the 10 cases with osteopenia. Osteopenia was present in 7 children who were not under CSI, but have received multiple trials of oral corticoids for severe exacerbations. CONCLUSIONS: Osteopenia was present in 52% of children that were under inhaled corticoids for more than 12 months and it correlated with the duration of CSI, but not with the daily dose. Systemic corticoid use is a significant cause of osteopenia in asthmatic children.

[Turner's syndrome, diagnosis and therapeutical approach]. / Sindromul Turner. Abordare diagnostica si terapeutica actuala.

Turner's Syndrome is a rare chromosomal disorder of females (1 : 2500) characterized by short stature and the lack of sexual development at puberty. Other physical features may include a webbed neck, heart defects, kidney abnormalities, and/or various other malformations. Diagnosis should be considered in individuals with primary or secondary amenorrhea and adult women with unexplained infertility, particularly when such individuals also are short in stature. In childhood, growth hormone therapy is standard to prevent short stature as an adult. Estrogen replacement therapy usually is required, but starting too early can compromise adult height. The most frequent, the possibility of a good diagnosis appears at puberty. The early diagnosis needs the karyotyping as screening method. The correct treatment, which is made on time, has a good mental and social life influence.

[Hemangioendothelioma in children]. / Hemangioendoteliom la copil.

The hemangioendothelium is a vascular tumor rarely seen in the pediatric practice. This case underlines the difficulty that lies in establishing a differential diagnosis with a localized adenopathy when this vascular tumor is developing inside a ganglionic region. The surgical treatment followed by interferon therapy determined a favorable evolution in this case because the hemangioendothelium is a vascular borderline tumor. Still, there are cases that recur, metastasize and have an evolution towards death.

[Apoptosis--basic regulatory mechanism of bone remodeling]. / Apoptoza--mecanism reglator de baza al remodelarii osoase.

Bone remodeling and regeneration take place permanently and are the result of a perfect balance between osteoclastic bone resorption and osteoblastic bone synthesis performed in the bone multicellular unit. Osteoblasts and osteoclasts differentiate from precursors under the influence of cytokines, growth factors mand cellular interactions controlled by adhesion molecules. The same growth factors and cytokines that promote bone cell differentiation have an important role in the prevention of cell death that is usually produced by apoptosis. The molecular mechanism of cytokines' and growth factors' actions is the activation of the pathway glicoprotein 130/Janus kinases/signal transductors and activators of transcription. The final step is the inhibition of cycline-dependent kinases, resulting in the inhibition of cell cycle and transition from G1 phase to S phase. Osteoblastogenesis and osteoclastogenesis are strongly bound together. Osteocytes are the most numerous bone cells, they have a long life, but ultimately die by apoptosis.