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Biomedical subjects

G Horev

Publications and source records attributed to G Horev.

At least 37 records · Page 2Linked to original sources

Iron overload following bone marrow transplantation in children: MR findings.

OBJECTIVE: The purpose of this study was to determine the incidence of post-transfusional iron overload in children after bone marrow transplantation by reviewing their magnetic resonance imaging (MR) findings. MATERIALS AND METHODS: We reviewed the abdominal MR studies of 13 children after autologous bone marrow transplantation. Nine of the children had also undergone MR prior to transplantation. Iron deposition in the liver, spleen and bone marrow was graded semi-quantitatively on both T1- and T2-weighted images. Serum ferritin levels and number of blood units given after bone marrow transplantation were recorded. RESULTS: None of the pre-transplantation MR studies revealed iron overload. After bone marrow transplantation, three children showed normal liver and spleen. Iron overload in the liver was noted in ten patients (77 %), six of whom also showed iron overload in the spleen (46 %) and five in the bone marrow (38.5 %). The degree of hepatic iron overload was correlated significantly and splenic iron overload was correlated weakly with the number of blood transfusions (P = 0.01 and P > 0.01, respectively), but neither was correlated with the serum ferritin level. CONCLUSION: Iron overload commonly accompanies bone marrow transplantation. The observed pattern of iron deposition, in which the spleen was uninvolved in 40 % of patients demonstrating iron overload, is not typical of post-transfusional hemochromatosis.

Abdomen↗

Tietze's syndrome in children and infants.

Tietze's syndrome, characterized by isolated swelling and tenderness of a costochondral junction, usually occurs in adults. We describe eight cases of Tietze's syndrome in children, four of them aged 1 year or less. The clinical aspects and laboratory and imaging findings should enable the clinician to recognize this benign entity, thereby avoiding invasive diagnostic procedures to rule out other conditions.

Child↗

Effect of acquisition of improved thermotolerance on the induction of heat shock proteins in broiler chickens.

The role of heat shock proteins (HSP) in the protection of cells from heat stress is well established. However, very little is known about their contribution to thermotolerance in the complexity of a whole homeotherm animal. Here we report on the analysis of protein synthesis in lung and heart muscle tissues of broiler chickens following exposure to high ambient temperature. Half of the flock was treated by an early age exposure to heat (conditioning), to improve thermotolerance. In contrast to what has been expected, lower levels of HSP induction was observed in the treated chickens. We suggest that 1) the induction of HSP in the heart and lung tissues of the whole animal correlates with the body temperature and 2) HSP response does not represent a part of the long-term mechanism that is evoked by the early age conditioning.

Aging↗

Visual pathway glioma: an erratic tumour with therapeutic dilemmas.

OBJECTIVE: Our experience in children with visual pathway glioma (VPG) was reviewed to delineate its clinical characteristics. DESIGN: The charts and imaging studies of 21 children with VPG who were followed up in our centre during the last 12 years were reviewed and summarised. RESULTS: VPG accounted for 13.1% of all brain tumours treated during this period. Sixty two per cent of the children with VPG had neurofibromatosis type 1 (NF-1). Among these, more than 60% were detected as part of routine work up. In some cases decreasing visual function preceded the appearance of the VPG on imaging studies. Tumour growth rate was markedly unpredictable. All treatment modalities employed led to tumour shrinkage and stabilisation for a variable period, but none was successful in totally eradicating the tumour. Complications were less severe after chemotherapy compared with radiotherapy. Three children died, none with NF-1, with a globular hypothalamic/chiasmatic tumour and accompanying electrolyte abnormalities. CONCLUSIONS: NF-1 is a favourable prognostic marker for VPG. Whenever possible a period of observation is necessary before treatment is initiated, during which time tumour size and visual function should be closely followed up; an untoward change in either of these is an indication for the start of treatment, preferably chemotherapy first. The combination of a globular hypothalamic/chiasmatic glioma and electrolyte abnormalities in a child without NF-1 are related to a poor prognosis.

Antibiotics, Antineoplastic↗

MR findings in hereditary isolated growth hormone deficiency.

PURPOSE: To describe the MR characteristics by which patients with hereditary isolated growth hormone deficiency (GHD) can be distinguished from patients with other types of GHD. METHODS: A total of 51 patients with GHD were examined prospectively with MR imaging. On the basis of familial occurrence of GHD and genetic analysis, 10 patients met the criteria for hereditary deficiency. In each case, the height of the pituitary gland, the presence and location of the posterior neurohypophysis, and the completeness of the stalk were recorded. The findings in the hereditary group were compared with those in the rest of the patients. RESULTS: In all 10 patients with hereditary GHD, the adenohypophysis, the neurohypophysis, and the stalk were normal. Of the other 41 patients, the height of the gland was normal in three (7%), the neurohypophysis was abnormal in all, and the stalk was truncated in all but two patients (95%). CONCLUSIONS: The subgroup of patients with hereditary GHD exhibited an anatomically normal pituitary-hypothalamic region. This is in contrast to the majority of patients with idiopathic GHD. MR imaging can contribute to the classification of patients with GHD.

Adolescent↗

Imaging of thoracoabdominal masses: the difficulty of compartmentalization.

Over the past 3 years we have encountered eight cases of masses in the thoracoabdominal junction or paradiaphragmatic region. To determine exact compartmentalization of the tumors prior to treatment, a detailed imaging evaluation, including conventional radiography, ultrasound, computerized tomography, and magnetic resonance imaging, was conducted. Despite these extensive examinations, we erred in three cases with regard to the compartmental limitation of the tumors. The imaging picture of para- or transdiaphragmatic penetration of masses can be very misleading and is at times resolved only at surgery.

Abdominal Neoplasms↗

Tracheomegaly in Brachmann-de Lange syndrome.

Brachmann-de Lange syndrome is a well-described congenital disorder. Skeletal anomalies and respiratory infections are its hallmarks. To the known imaging signs, the authors add tracheomegaly, which has not been reported so far.

Child↗

The natural history of osteonecrosis of the femoral head in children and adolescents who have Gaucher disease.

We reviewed the cases of eight patients (thirteen hips) with Gaucher disease who had had osteonecrosis of the femoral head and had been managed with bed rest and non-weight-bearing with crutches only in the symptomatic stage of the bone crisis. The mean age of the patients at the onset of the first crisis in each hip was ten years (range, six to fourteen years). The mean age at the most recent follow-up examination for the six living patients (ten hips) was twenty-three years (range, nineteen to thirty-three years). The Mose rating was good for one hip, fair for two, and poor for seven. Despite the over-all unfavorable radiographic ratings, the six patients were asymptomatic and did not need assistance with daily activities. There is no known treatment that effectively prevents the development of deformities of the femoral head. Thus, we recommend symptomatic management for osteonecrosis of the femoral head in Gaucher disease with bed rest and analgesics followed by non-weight-bearing on the involved limb, if it makes the patient more comfortable, during the symptomatic stage of bone crisis.

Adolescent↗

A novel 29-kDa chicken heat shock protein.

The family of small heat shock proteins is the more variable among the highly conserved superfamily of heat shock proteins (HSP). Using a metabolic labeling procedure with tissue explants, we have detected in chickens a new member of the small HSP family with an apparent molecular weight of 29-kDa. This protein was induced in broiler chickens' heart muscle and lungs following an in vivo heat stress. The 29-kDa band appears after 3 h of heat stress, much later than the induction of HSP 90, HSP 70, and HSP 27. The late onset of induction suggests that HSP 29 plays a more specific role of a "second stage defense protein".

Animals↗

Central precocious puberty: evaluation by neuroimaging.

To evaluate the incidence of abnormal intracranial findings in children with central precocious puberty, 62 children (51 girls, 11 boys) were examined by computerized tomography and/or magnetic resonance imaging (MRI) of the brain. Forty-four had normal examinations; 18 (11 girls, 7 boys) showed intracranial pathologies, including hamartoma of the tuber cinereum (8 cases), parenchymal loss (3 cases), hypothalamic-chiasmatic lesions (2 cases), lesions of the corpus callosum (2 cases), suprasellar cyst (1 case), and pineal cyst and mesiotemporal sclerosis (1 case each). Based on the correlation between the clinical and the imaging results of this series, the authors recommend MRI as the imaging method of choice in the investigation of precocious puberty.

Brain Diseases↗

Morning Glory fundus anomaly, coloboma of the optic nerve, porencephaly and hydronephrosis in a newborn infant: MCPH entity.

The association of Morning Glory anomaly with intracranial pathology has been rarely described and seems to be a distinct one. A 41-week full-term infant presenting with Morning Glory anomaly and multiple major and minor anomalies with a normal karyotype is described. Right Parieto-occipital porencephaly with mild hydrocephaly of lateral ventricle, right brain atrophy and enlarged thalami were observed by repeated ultrasound, computed tomography and magnetic resonance imaging examinations. Fundoscopy, visual evoked response and electroretinography revealed bilateral colobomas of the optic nerve, bilateral Morning Glory optic disc anomaly, severe exotropia and medial recti paralysis. Non-obstructive, non-refluxing left hydronephrosis and left hydroureter were diagnosed by renal investigations. To the best of our knowledge the association of the described malformations has never been previously reported. The clinical and nosological significance of this new entity is discussed.

Adult↗

[Subdural empyema complicating sinusitis].

Paranasal sinusitis complicated by subdural empyema (SDE) in a 14-year-old boy is described. He presented with fever, headache and vomiting. X-rays revealed left maxillary and bilateral frontal sinusitis. While in the emergency room he had seizures; CT-scan showed an air leak adjacent to the right frontal sinus. Despite intravenous antibiotics, left hemiparesis developed and repeat CT showed interhemispheric SDE. Frontal craniotomy was performed and a large amount of purulent material was drained. Recovery followed 4 weeks of intravenous antibiotic treatment without neurologic sequelae. SDE is a rare complication of paranasal sinusitis and constitutes approximately 20% of all localized intracranial infections. Clinical features include fever, headache, vomiting, convulsions and neurologic deficits. Modern imaging methods and combined surgical and antibiotic treatment have lowered mortality to 5-10%. A high index of suspicion is important for early diagnosis and successful treatment.

Adolescent↗

A rare coexistence of a multicentric hepatic hemangioendothelioma with a large brain hemangioma in a preterm infant.

A preterm infant with a rare association of a multicentric hepatic hemangioendothelioma (MHH) and a large brain hemangioma is described. The imaging investigations and their findings are discussed. It is recommended that whole-body radionuclide imaging with 99mTc-labeled red blood cells is employed immediately following ultrasonography to confirm the suspected diagnosis of MHH and to detect other unsuspected hemangiomas.

Brain Neoplasms↗

The pattern of bone disease in transfusion-dependent thalassemia major patients.

Twenty-eight patients with thalassemia major were treated with frequent blood transfusions for 10-25 years. Eleven (39%) had radiographic signs of osteoporosis, and four (14%) presented with fractures. Keeping hemoglobin level above 9.0 g/dl reduced osteoporosis and the incidence of fractures but did not prevent them. Osteonecrosis of the femoral head and distal femur, not previously reported, was noted in two patients.

Adolescent↗

Spinal involvement in children and adolescents with Gaucher disease.

Nineteen patients with type I Gaucher disease with spinal involvement first diagnosed during childhood, were followed for 2 to 24 years (average 9 years), and their clinical and radiologic history reviewed. Patients presented with three types of pain: mild pain that was defined as nonspecific, severe pain due to bone crisis, and pain associated with vertebral collapse. Collapse of vertebra occurred gradually anywhere along the thoraco-lumbar spine, and usually more than one vertebra was involved. In three patients, rectangular collapse was noted. Six patients suffered from central vertebra collapse, and two from anterior wedge compression. Further vertebral collapse with signs of root and cord compression developed in three patients as they grew. Awareness to the possibility of progressive vertebral collapse and periodic follow-up might prevent severe complications.

Adolescent↗

Bronchiectasis in children: assessment by CT.

The prevalence of bronchiectasis (BR), in general, is decreasing, yet the disease can produce significant morbidity in children. In the pediatric age group the classical investigation by bronchography implies general anaesthesia, thus carrying an additional risk of complications. CT has proved highly accurate in the diagnosis of BR in adults. It is also considered a reliable modality for the diagnosis of BR in children. This conclusion was reached by analysing the radiographic and the CT findings in 40 children with the clinical suspicion of BR in 25 of whom the CT examination was positive. Nine patients of this last group had bronchography as well. There was complete correlation in the diagnosis and location between the CT and the bronchographic findings. Thus, it seems that the occurrence of this disease is still high in the pediatric population in the appropriate clinical and radiological setting. The imaging evaluation of BR should include chest radiographs, computerized tomography and, if surgery is planned, bronchography as well.

Adolescent↗