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Biomedical subjects

Liora Kornreich

Publications and source records attributed to Liora Kornreich.

At least 19 recordsLinked to original sources
PubMed2006

Duplex sonographic findings in children with torsion of the testicular appendages: overlap with epididymitis and epididymoorchitis.

BACKGROUND AND PURPOSE: Torsion of the testicular appendages (TTA) is the most common cause of acute scrotum in children, and yet there are only few dedicated studies of the imaging findings. OBJECTIVE: To review our experience with the use of duplex sonography in children with TTA and to evaluate if sonography can successfully distinguish TTA from epididymoorchitis. METHODS: We reviewed the medical files and imaging findings of 29 children aged 0.7 to 13.9 years (mean, 6.5 years) with a diagnosis of TTA based on testicular exploration who were evaluated preoperatively with duplex sonography. RESULTS: A tender upper pole nodule, the typical sign of TTA, was palpated in only 2 (6.9%) children. Duplex sonography demonstrated an extratesticular upper pole nodule in 9 (31%) children. Secondary inflammatory changes included hydrocele in 22 (75.9%), enlarged epididymis in 22 (75.9%), scrotal wall edema in 16 (55.2%), and swollen testis in 9 (31%) children. Fourteen (48%) children had inflammatory changes with no evidence of an extratesticular nodule. CONCLUSION: Duplex sonography findings of secondary inflammatory changes in the absence of evidence of an extratesticular nodule may suggest an erroneous diagnosis of epididymitis or epididymoorchitis in children with TTA.

Acute Disease↗
PubMed2006

Idiopathic granulomatous encephalitis mimicking malignant brain tumor.

Idiopathic granulomatous encephalitis is a rare disorder of unknown etiology, undetermined treatment, and often grave prognosis. This article describes a 4-year-old female who presented with a single focal febrile convulsion followed a few weeks later by right-sided hemiparesis. A huge infiltrative cerebral mass tumor was found which proved to be a granuloma on histologic study. Despite a thorough evaluation, including tissue studies and search for an infectious agent, no etiology could be identified, and the final diagnosis was idiopathic granulomatous encephalitis. Recurrent resections and high-dose steroid treatment failed to control the process, and the patient died of disease 6 months after presentation. Evaluation and treatment of idiopathic granulomatous encephalitis should be aggressive, and the possibility of chemotherapy and perhaps even radiotherapy should be considered if there is no response to steroids.

Anti-Bacterial Agents↗
PubMed2006

Treatment of neuroblastoma using the fused imaging guided radiotherapy (FIGURA) system.

PURPOSE: The purpose of this study was to describe our department's experience with the fused imaging-guided radiotherapy (FIGURA) system for planning radiation treatment of high-risk neuroblastoma. PATIENTS AND METHODS: Between 1999 and 2002, 11 patients received radiation therapy as consolidation after chemotherapy in 9 and for palliation in 2. Diagnostic metaiodobenzylguanidine (MIBG) imaging was used, which is specific for neuroblastoma, to identify the residual tumor, followed by computed tomography scanning in the radiation treatment position. The FIGURA software fused the images obtained by the 2 modalities and transferred the result to a 3-dimensional radiation treatment planning system. Radiation was delivered at a total dose of 25.2 Gy according to the FIGURA. RESULTS: Five patients achieved complete remission and 2 partial remission; 3 were stabilized. One child with a highly rapid progressive course died of the disease. CONCLUSION: FIGURA is a new, feasible technique for defining target volumes. By using standard hospital equipment, it is possible to treat residual disease identified by sensitive metaiodobenzylguanidine imaging and localized with the anatomic computed tomography scan. Treating a more accurate target volume spares normal tissue and organs and minimizes side effects.

Child↗
PubMed2005

Role of MRI in the management of children with diffuse pontine tumors: a study of 15 patients and review of the literature.

BACKGROUND: Pontine tumors carry the worst prognosis of all brain tumors. In most cases, the diagnosis is based solely on MR imaging, without biopsy. OBJECTIVE: To describe the MR findings of pontine tumors at diagnosis and during follow-up and correlate those with prognosis and to assess the value of MR imaging in patient management compared to clinical evaluation. MATERIALS AND METHODS: Ninety-one MR scans of 15 children with diffuse pontine tumors were reviewed at diagnosis and during follow-up. The parameters analyzed were as follows: tumor extent, area, and volume; encasement of the basilar artery; presence of exophytic component; necrosis; cysts; hydrocephalus; and intensity and enhancement. Findings were correlated to length of progression-free and overall survival. Trends of amelioration or worsening on imaging were compared with the clinical findings. RESULTS: Median length of progression-free survival was 10 months, and median survival was 20 months. Only hydrocephalus at presentation was associated with shorter progression-free survival (P=0.02). On the last examination of each patient, the craniocaudal diameter was significantly greater than at diagnosis (P=0.03). The concordance between the imaging and the clinical findings was good. CONCLUSION: MR is the mainstay for the diagnosis and management of pontine tumors. Cranial growth seems to be an ominous sign. However, the prognostic value of MR is limited. MR findings correlate well with the clinical examination.

Adolescent↗
PubMed2005

Wandering spleen--the challenge of ultrasound diagnosis: report of 7 cases.

PURPOSE: To summarize our experience with sonographic diagnosis of wandering spleen in children and assess for the typical sonographic findings of wandering spleen, complications, and possible diagnostic pitfalls. METHODS: We identified all pediatric patients from 1998-2003 with a surgically confirmed diagnosis of wandering spleen. All sonographic examinations were reviewed for splenic position, size, echotexture, and parenchymal blood flow. RESULTS: Seven children were identified with a mean age of 9.7 years (range 4.2-15.3 years). All presented with abdominal pain. Abdominal sonography, performed in all children demonstrated a low position of the spleen (n = 6), splenomegaly (n = 4), and absence of parenchymal flow in the three patients with splenic torsion and infarction. The diagnosis was made preoperatively via sonography in five children; three required repeated hospital admissions before the correct diagnosis was established. Complications occurred in five patients (gastric obstruction [n = 1], splenic infarction [n = 3], and recurrent pancreatitis [n = 1]). CONCLUSION: The most specific sonographic finding for wandering spleen is low position of the spleen. However, if the spleen regains its normal or near-normal position, the diagnosis may be missed and the condition may recur, and result in complications.

Abdominal Pain↗
PubMed2005

Acute mastoiditis and osteomyelitis of the temporal bone.

OBJECTIVE: Acute mastoiditis becomes clinically significant when infection spreads through the periosteum and induces periosteitis. This study describes an atypical complication of acute mastoiditis: osteomyelitis of the temporal bone. PATIENTS AND METHODS: The study sample included all patients admitted for acute mastoiditis between September 2001 and December 2003 who had symptoms, signs and imaging findings of osteomyelitis of the temporal bone beyond the mastoid area. The files were reviewed for diagnosis, work-up, radiographic findings and treatment. RESULTS: The study group included 6 of the 120 patients treated for acute mastoiditis. In four children (66%), the diagnoses of acute otitis media and acute mastoiditis were made simultaneously at admission. Ear cultures yielded coagulase-positive Staphylococcus in three patients, Bacteroides in two, multiple organisms in two, S. pneumoniae in one, and no growth in two. Complications were suspected if there was a lack of improvement in symptoms and signs, or in cases of skin involvement over the temporal bone beyond the area of the mastoid in accordance with imaging findings. Computerized tomography demonstrated temporal bone absorption beyond the mastoid area (squama and/or petrous bones) in all children, suspected sinus vein thrombosis in two, and suspected epidural abscess in one. All children were treated with at least cortical mastoidectomy and insertion of ventilation tubes. Revision mastoidectomy was performed in three children in whom no improvement was noted and imaging suggested other complications. CONCLUSION: The present study describes an unusual complication of acute mastoiditis-osteomyelitis of the temporal bone beyond the mastoid framework. The disorder is characterized by a failure to respond both locally and systemically to accepted medical and surgical therapy, persistent fever and high levels of inflammatory markers, and computerized tomography findings of temporal bone destruction. Treatment includes broad-spectrum antibiotics and at least cortical mastoidectomy. Prognosis is good.

Acute Disease↗
PubMed2005

Task switching after cerebellar damage.

The authors of this study investigated task switching following cerebellar damage. The study group consisted of 7 children and adolescents (M age=13.8 years) who underwent surgical removal of a benign posterior fossa tumor. They were tested at a sufficient interval after surgery (M lag=6.13 years) for restoration of normal cognitive skills and intelligence. Although all showed normal learning of the task compared with control participants, when rapid behavioral changes were required (short preparation time), they exhibited behavioral rigidity manifested by enhanced switching cost. These results are in line with another study on serial reaction time with the same patients (A. Berger et al., in press). They have important implications for our understanding of the cognitive sequelae of early cerebellar damage as well as the involvement of the cerebellum in task switching.

Adolescent↗
PubMed2005

Prenatal imaging of fetal hepatoblastoma.

A detailed sonographic at 37 weeks of gestation performed due to non-reassuring fetal monitor of a breech presentation fetus detected a solid right lobe hepatic tumor. Prenatal gray scale and Doppler sonography suggested a diagnosis of hepatoblastoma which was confirmed postnatally. Differential ultrasonographic diagnosis of fetal hepatic tumors is discussed.

Adult↗
PubMed2005

Serum ferritin level as a predictor of impaired growth and puberty in thalassemia major patients.

OBJECTIVE: Previous studies suggested that in patients with thalassemia major, initiating deferoxamine (DFO) therapy before puberty can prevent iron-induced failure of growth and puberty. However, early initiation of chelation has also been associated with DFO toxicity. The aim of this retrospective study was to determine the prevalence rates of endocrine complications and DFO bone toxicity in our thalassemia major patients and to correlate them with the degree of iron chelation. METHODS: Thirty-nine patients with thalassemia major were followed for a median of 16.3 yr (range 2-28). Individual mean serum ferritin level during the study period was calculated using repeated annual measurements. Bone DFO toxicity was assessed by wrist and spine radiographs; endocrine dysfunction by anthropometric measurements and pubertal stage; and hypogonadotropic hypogonadism by lack of luteinizing hormone response to gonadotropin-releasing hormone. RESULTS: Chelation therapy was initiated at median age 4.9 yr. Mean serum ferritin level during the study period was 2698 +/- 1444 ng/mL. Hypogonadism was noted in 59% of the patients who reached pubertal age, and short stature was found in 36% of patients who reached final height. Mean ferritin level of 2500 ng/mL during puberty was the cut-off for hypogonadism, and ferritin level of 3000 ng/mL during prepuberty was the cut-off for final short stature. None of the patients who attained final height had signs of DFO bone toxicity. CONCLUSIONS: High serum ferritin levels during puberty are a risk factor for hypogonadism, and high serum ferritin levels during the first decade of life predict final short stature. It remains to be determined whether improving chelation by earlier initiation of DFO or by the combined use of DFO and deferiprone will lead to better growth and sexual development without DFO toxicity.

Adolescent↗
PubMed2005

Complications of mastoiditis in children at the onset of a new millennium.

The aim of the present study was to review our recent experience in the diagnosis and treatment of acute mastoiditis and its complications in a single tertiary-care, university-affiliated pediatric center. Ninety-eight children with 101 episodes of acute mastoiditis were included in the study. The mean interval from onset of illness to mastoiditis was 4.5 days. Ear cultures most often grew Streptococcus pneumoniae and Pseudomonas aeruginosa (23.7% each). Complications occurred in 15.8% of episodes. The only factor differentiating children with and without complications was white blood cell count. These findings indicate that acute mastoiditis not only is a complication of prolonged infection of the middle ear, but may also present as an acute infection of the mastoid bone that can progress within 48 hours. The complication rate remains high, and antibiotic treatment at the onset of symptoms does not prevent complications. A high white blood cell count on admission may serve as a predictive factor of complicated cases.

Acute Disease↗
PubMed2005

Familial cognitive impairment with ataxia with oculomotor apraxia.

Ataxia with oculomotor apraxia is an autosomal recessive inherited disease characterized by childhood onset of progressive cerebellar ataxia, oculomotor apraxia, and progressive motor peripheral neuropathy. The mean age at onset is approximately 4.7 years, with oculomotor apraxia appearing a few years later. Diagnosis is based on molecular genetic analysis for mutations of the aprataxin (APTX) gene (chromosome 9p13.1; ataxia with oculomotor apraxia 1). Ataxia with oculomotor apraxia 2 is caused by an unknown gene mutation at locus 9q34. We describe two siblings, born to consanguineous parents, who had clinical features of cerebellar ataxia, tremor, dysarthria, oculomotor apraxia, and motor peripheral neuropathy. Brain magnetic resonance imaging showed cerebellar atrophy and mild brainstem atrophy. Electromyography showed signs of axonal neuropathy. The molecular genetic analysis demonstrated the APTX mutation W279X at locus 9p13.3 (ataxia with oculomotor apraxia 1 disease), and psychologic studies showed mild cognitive impairment. We suggest that mentation can be compromised in ataxia with oculomotor apraxia 1.

Adolescent↗
PubMed2005

Thiamine deficiency in infants: MR findings in the brain.

BACKGROUND AND PURPOSE: Thiamine deficiency is extremely rare in infants in developed countries. To our knowledge, its MR findings in the brain have not been reported. The purpose of this study was to investigate the brain MR findings in infants with encephalopathy due to thiamine deficiency. METHODS: The study group included six infants aged 2-10 months with encephalopathy who had been fed with solely soy-based formula devoid of thiamine from birth. All underwent MR evaluation at admission and follow-up (total of 14 examinations). In one patient, MR spectroscopy (MRS) was performed. RESULTS: In five patients T2-weighted, fluid-attenuated inversion recovery, or proton-attenuated sequences showed bilateral and symmetric hyperintensity in the periaqueductal area, basal ganglia and thalami. Five had lesions in the mammillary bodies, and three, in the brain stem. In all six patients, the frontal region (cortex and white matter) was clearly involved. At presentation, MRS of the periaqueductal area showed a lactate doublet. On long-term follow-up, three of four patients had severe frontal damage; in two, this occurred as part of diffuse parenchymal loss, and in one, it was accompanied by atrophy of the basal ganglia and thalami. CONCLUSION: Thiamine deficiency in infants is characterized by involvement of the frontal lobes and basal ganglia, in addition to the lesions in the periaqueductal region, thalami, and the mammillary bodies described in adults. MRS demonstrates a characteristic lactate peak.

Brain↗
PubMed2005

Motor and non-motor sequence learning in children and adolescents with cerebellar damage.

Cerebellar involvement in motor and non-motor sequence learning was examined with serial reaction time tasks (SRT). Our sample consisted of 8 children and adolescents who had undergone surgical removal of a benign posterior fossa tumor (PFT) during childhood. None of them had undergone chemotherapy or cranial radiation therapy (CRT). Ages ranged from 1-11 years at surgery and 9-17 years at testing. The children were tested not earlier than 2.5 years after surgery (M = 5.9 years), enabling brain plasticity and recovery of functions. Their performance was compared with a matched control sample. The PFT group was not impaired in the implicit learning of sequences, as reflected in their performance in blocks with a repeated sequence, both before and after a random block. However, in the perceptual task, their performance deteriorated more than that of the control group when a random block was introduced, suggesting that it was more difficult for the patients to respond flexibly or change their response set when encountering changing task demands. These results are in line with another study by our group on task switching with the same patients.

Adolescent↗
PubMed2004

Clinical and sonographic criteria of acute scrotum in children: a retrospective study of 172 boys.

BACKGROUND: Diagnosis of testicular torsion in children is challenging, as clinical presentation and findings may overlap with other diagnoses. OBJECTIVE: To define the clinical and ultrasound criteria that best predict testicular torsion. MATERIALS AND METHODS: The records of children hospitalized for acute scrotum from 1997 to 2002 were reviewed. The clinical and ultrasound findings of children who had a final diagnosis of testicular torsion were compared with those of children who had other diagnoses (torsion of the testicular appendix, epididymitis, and epididymo-orchitis). RESULTS: Forty-one children had testicular torsion; 131 had other diagnoses. Stepwise regression analysis yielded three factors that were significantly associated with testicular torsion: duration of pain < or =6 h; absent or decreased cremasteric reflex; and diffuse testicular tenderness. When the children were scored by final diagnosis for the presence of these factors (0-3), none of the children with a score of 0 had testicular torsion, whereas 87% with a score of 3 did. The ultrasound finding of decreased or absent testicular flow had a sensitivity of 63% and a specificity of 99%. Eight of ten children with testicular torsion and normal or increased testicular flow had a coiled spermatic cord on ultrasound. CONCLUSION: We suggest that all children with acute scrotal pain and a clinical score of 3 should undergo testicular exploration, and children with a lower probability of testicular torsion (score 1 or 2) should first undergo diagnostic ultrasound. Because the presence of testicular flow does not exclude torsion, the spermatic cord should be meticulously evaluated in all children with acute scrotum and normal or increased testicular blood flow.

Acute Disease↗
PubMed2004

Prothrombotic factors in children with otitis media and sinus thrombosis.

OBJECTIVE: Venous sinus thrombosis (VST) is the second most common intracranial complication of acute otitis media (AOM). There is some evidence that hereditary and acquired prothrombotic disorders are risk factors for VST. The aim of the present study was to evaluate whether children with AOM complicated VST have a prothrombotic tendency, which might have important therapeutic and preventive implications. STUDY DESIGN: Retrospective. METHODS: The files of children hospitalized at Schneider Children's Medical Center of Israel, a tertiary referral center, from 1999 to 2002, because of AOM complicated by acute mastoiditis and VST were reviewed. All children underwent laboratory work-up for hypercoagulability. RESULTS: Seven children met the study criteria. Of these, five had prothrombotic disorders, namely elevated levels of lipoprotein apolipoprotein (Lp[a]) (n = 4), antibodies to beta 2-glycoprotein and to cardiolipin (markers of antiphospholipid syndrome) (n = 4), and heterozygosity for factor V Leiden mutation (n = 1). One child had three abnormalities, two children had two abnormalities, and two children had one abnormality. CONCLUSIONS: A prothrombotic tendency may exist in children with AOM complicated by mastoiditis and VST. Further studies are needed to evaluate its extent.

Acute Disease↗
PubMed2003

Epilepsy associated with pediatric brain tumors: the neuro-oncologic perspective.

The aim of the study was to correlate the onset of epilepsy with the disease stage in children with brain tumors through treatment and follow-up in the oncologic department. The study sample consisted of a heterogenous group of 219 children who were aged 6 months to 11 years, manifested brain tumors, and had been treated and monitored in the Department of Pediatric Oncology of the Schneider Children's Medical Center of Israel since 1991. The overall rate of epilepsy was 14.6%, which rose to 38% in those with cortical tumors. Two major causes of epilepsy were evident: tumor-related and treatment-related. The first group could be further divided into epilepsy starting at or before diagnosis of brain tumor, epilepsy associated with tumor progression, and epilepsy starting at end-stage disease. The second group could be divided into epilepsy caused by radiation damage to the brain and epilepsy related to another postoperative state. The data emphasize the significance of striving for complete tumor resection and the potential damage from the use of radiotherapy to the brain. The authors suggest that a change in local neurotransmitter balance may be the mechanism underlying tumor-related epilepsy.

Adolescent↗
PubMed2003

Pituitary size in patients with Laron syndrome (primary GH insensitivity).

OBJECTIVE: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron syndrome, leads to an increase in the size of the pituitary gland. METHODS: Eleven patients (six females, five males) with Laron syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36-68 Years and two children, a 4-Year-old boy and a 9-Year-old girl. The latter patient had been treated with IGF-I (150-180 mg/kg per day) since the age of 3 Years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. RESULTS: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary-hypothalamic region were detected. CONCLUSION: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary-hypothalamic region in Laron syndrome is normal.

Adult↗