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Biomedical subjects

Y Uziel

Publications and source records attributed to Y Uziel.

13 recordsLinked to original sources

Cerebral vein thrombosis in childhood systemic lupus erythematosus.

We describe three cases of cerebral vein thrombosis (CVT) in girls with systemic lupus erythematosus. Severe, persistent, unremitting headache was a common manifestation. In the first patient, although the clinical features were suggestive of CVT, the diagnosis was delayed and she had a significant cerebral infarct. In the other two patients the diagnosis was made earlier and led to more rapid treatment; the institution of early therapy may have prevented further sequelae. The CVT was diagnosed in all patients with a combination of computed tomography and magnetic resonance imaging studies without the need for angiography. All patients were treated for their underlying systemic lupus erythematosus and with anticoagulation. All are receiving long-term low doses of warfarin and have not had any recurrences.

Adolescent

Scleroderma in children.

Childhood scleroderma may present in a variety of clinical forms that differ in clinical presentation, extracutaneous features, clinical course, and outcome. All include hardening of the skin as a major feature. This article reviews these various entities, focusing on primarily the clinical features. In addition, current concepts regarding pathogenesis and treatment are discussed.

Adolescent

Toxocariasis: a diagnostic dilemma.

Visceral larva migrans (VLM) is endemic in Israel; therefore, a positive serology is not unusual in our general population. The clinical manifestations of the disease may mimic other clinical conditions. Sometimes life-threatening disorders have to be considered in the differential diagnosis. This diagnostic dilemma can be solved occasionally only by histological examination. We report two patients, one with a lymphoma-like presentation and the other with an intraocular tumoral mass suggesting retinoblastoma, and discuss the diagnostic difficulties.

Child, Preschool

Serum levels of soluble interleukin-2 receptor. A marker of disease activity in localized scleroderma.

OBJECTIVE: To determine whether circulating serum levels of soluble interleukin-2 receptor (sIL-2R) are elevated in patients with localized scleroderma, and if levels of sIL-2R can differentiate between active and inactive disease. METHODS: Seventeen patients with localized scleroderma were categorized by overall physician assessment into active, inactive, and indeterminate groups, according to disease activity. Serum sIL-2R levels were analyzed and correlated with disease activity. RESULTS: The mean sIL-2R level was significantly higher (P = 0.005) in those with active disease (1,675 +/- 823 units/ml) than in those with inactive disease (722 +/- 218 units/ml). CONCLUSION: Serum sIL-2R levels are elevated in patients with localized scleroderma. When present, elevated sIL-2R levels appear to be able to differentiate active from inactive disease. This fact also suggests cell-mediated immune activation in this condition. Further serial studies are required to assess the value and sensitivity of sIL-2R levels in measuring changes in disease activity.

Adolescent

Localized scleroderma: imaging features.

Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with "en coup de sabre" facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients.

Adolescent

Localized scleroderma in childhood: a report of 30 cases.

Localized scleroderma (LS), a rare disease that occurs primarily in the pediatric age group, differs from systemic sclerosis (SSc) in that it is usually limited to the skin and subcutaneous tissue and is only rarely associated with systemic manifestations. The authors' experience with pediatric LS seen in 30 patients at a tertiary care center was reviewed: 26 had linear scleroderma, 19 on an extremity and 7 on the face; 3 had morphea; and 1 had generalized morphea. Antinuclear antibodies were present in 76% and rheumatoid factor in 39%. Five of 19 patients with linear scleroderma that involved an extremity had growth failure in that limb, and 1 required surgery. Sclerodermatous involvement over a joint resulted in limited range of movement in 6 patients, and 1 required surgery. One of the 30 patients developed SSc and polymyositis. There was difficulty in evaluating disease activity and hence in evaluating treatment. This experience with a large patient population suggests that LS, although usually a self-limiting disease, can result in significant morbidity.

Adolescent

Nose tapping test inducing a generalized flexor spasm: a hallmark of hyperexplexia.

The present report describes a benign disorder of neonates or young infants presenting with generalized hypertonicity accompanied by brisk muscle stretch reflexes, intermittent clonus, and exaggerated startle response. This condition is termed hyperexplexia, and may be either familial or sporadic. Two affected families are reported: Two sisters and their mother are involved in the first family; in the second family, where the parents are asymptomatic first degree cousins, all three siblings suffer from hyperexplexia of various severity. Nose tapping in infants of affected families induced a uniform reaction of facial twitching accompanied by head extension, and a generalized flexor spasm, all of which may be a hallmark of hyperexplexia. Severely hypertonic infants were treated with small doses of benzodiazepines and improved markedly, all becoming asymptomatic by two years of age. Unnecessary investigation and treatment may be avoided by sufficient awareness of possible hyperexplexia.

Child, Preschool

Cat-scratch bacillus and Streptococcus pneumoniae causing submandibular suppurative adenitis and acute glomerulonephritis.

Acute cervical adenitis is a frequent problem in pediatrics. It is occasionally followed by acute postinfectious glomerulonephritis, which has a well-defined set of clinical and histological manifestations. We present two rare cases of acute postinfectious glomerulonephritis, one associated with Streptococcus pneumoniae and the second with cat-scratch disease. Cultures of material from the lymph nodes yielded S. pneumoniae type 15 in case 1. A pleomorphic gram-negative micro-organism, identified by silver stain as cat-scratch bacillus, was found in case 2.

Cat-Scratch Disease

[Hospitalization policy following minor head injuries].

Of 44 patients admitted with head injuries during 1980-84, 316 (72%) arrived fully conscious with normal neurological status and no X-ray evidence of skull fracture. These cases were admitted for observation, but in none was there any untoward development. On analysis of the cases that developed complications and in the light of the literature, we conclude that it is possible to define a group at low risk for developing complications following head injury. These patients could safely be discharged from the emergency room without admission for observation. This group included 176 (40%) of those admitted and fulfilled the following criteria. They arrived fully conscious, there was no recurrent vomiting, neurological examination was normal, there was no X-ray evidence of skull fracture, and they were between 16-70 years of age. However, these cases should only be discharged from the emergency room after a physician gives oral and written instructions to a responsible adult who accompanies the patient. Such a policy would result in considerable saving of resources by reducing admissions for observation of those at low risk for developing complications.

Adolescent

Acute carbamazepine poisoning and hyponatremia.

A case of acute carbamazepine poisoning is described, which demonstrates all the immediate side effects of poisoning. Emphasis is placed on water intoxication and hyponatremia as the main cause of irreversible cerebral damage.

Carbamazepine