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Biomedical subjects

L Harel

Publications and source records attributed to L Harel.

At least 19 recordsLinked to original sources
PubMed2000

Carbamazepine-induced Stevens-Johnson syndrome treated with IV steroids and IVIG.

A 17-year-old female is presented who developed antiepileptic drug hypersensitivity syndrome after treatment with carbamazepine. The initial diagnoses were idiopathic thrombocytopenic purpura and toxic shock syndrome. The patient was treated with intravenous immunoglobulin and intravenous steroids. After a severe climax on days 2-4 she recovered completely and was discharged on the eighth day of hospitalization. Although we do not have direct proof, we believe that these treatment modalities, especially the IVIG, shortened and ameliorated the clinical course of the disease.

Adolescent↗
PubMed2000

Raynaud's phenomenon as a manifestation of parvovirus B19 infection: case reports and review of parvovirus B19 rheumatic and vasculitic syndromes.

Infection with human parvovirus B19 is manifested as erythema infectiosum, transient aplastic crisis, or hydrops fetalis. Rheumatic manifestations include arthropathy and various vasculitic syndromes. Isolated Raynaud's phenomenon due to parvovirus B19 has never been described. We report on 2 previously healthy sisters with new-onset Raynaud's phenomenon accompanied by severe generalized polyarthralgia. A full workup was negative, except serology for parvovirus B19, which was positive. All symptoms gradually subsided within 3-5 months, and no recurrence has been noted during the 3 years since onset. We review all the studies in the English-language literature on parvovirus B19-induced rheumatic and vasculitic syndromes. We hypothesize that the pathogenesis of Raynaud's phenomenon in our patients involved immune-mediated endothelial damage leading to platelet activation and vasoconstriction. We recommend that in cases of unexplained Raynaud's phenomenon, serology for parvovirus B19 be included in the evaluation.

Adolescent↗
PubMed1999

Meyer dysplasia in the differential diagnosis of hip disease in young children.

OBJECTIVES: To describe a rare developmental disorder of the femoral capital epiphysis in infants and children that is often misdiagnosed and to suggest an evaluation protocol to differentiate it from other hip problems. DESIGN: Case series. SETTING: Tertiary care center. SUBJECTS: Five consecutive patients referred for evaluation of acute onset of limping between January 1990 and December 1997. INTERVENTION: All clinical and imaging data were collected. RESULTS: Two of the 5 patients were initially diagnosed as having osteomyelitis and 3 as having Perthes disease. The diagnosis of Meyer dysplasia was confirmed by plain film of the pelvis, a negative bone scan, or normal bone marrow findings on magnetic resonance imaging. The limping resolved without treatment in all patients within 1 to 3 weeks. CONCLUSIONS: Meyer dysplasia is a benign condition that should be included in the differential diagnosis of hip disease in infants and children. Awareness of this condition may prevent unnecessary hospitalization and treatment.

Child, Preschool↗
PubMed1999

Childhood Behçet's disease: clinical features and comparison with adult-onset disease.

OBJECTIVE: To study the clinical spectrum of Behçet's disease (BD) in childhood, in comparison to adult-onset disease. METHODS: Nineteen children, who fulfilled disease criteria up to the age of 16 yr, were studied. The results were compared to those of 34 adult patients with BD. An activity index and severity score were calculated for both study groups. RESULTS: The mean age of disease onset was 6.9+/-3.9 yr, similar ages of onset were found in males and females. The clinical spectrum of childhood BD resembled that of adult disease; however, the prevalence of certain manifestations was different between children and adults. Children with BD had significantly less genital ulcers, less vascular thromboses and more non-specific gastrointestinal symptoms, as well as central nervous system involvement and arthralgia. A relatively high prevalence of uveitis was found in childhood BD. The activity index and severity score were significantly lower in children than in adults. CONCLUSION: Our results point to a similar systemic expression of BD in children and adults; however, the disease seems to run a less severe course in children.

Adolescent↗
PubMed1999

Seasonal variation in systemic onset juvenile rheumatoid arthritis in Israel.

OBJECTIVE: To determine whether there is a seasonal peak onset of systemic juvenile rheumatoid arthritis (SOJRA) suggestive of an infectious etiology. We examined the seasonal variability of SOJRA in Israel. METHODS: A multicenter retrospective chart review of 59 patients with SOJRA, enrolled from 10 rheumatology units or pediatric departments in Israel. All patients met defined criteria of SOJRA. RESULTS: Fifty-nine patients (31 female, 28 male) were followed from 1982 to 1997. Their mean age was 7.1 +/- 4.3 years (range 0.9-16). Forty-six were Jewish and 13 were Arabs or of Bedouin origin. Eighteen patients (31%) had disease onset in the winter, 16 (27%) in the spring, 12 (20%) in the summer, and 13 (22%) in the fall. Twenty-eight patients had a monophasic disease subtype, while 31 had a chronic or cyclic subtype. The seasonal onset in the patients with the monophasic type versus the chronic or the cyclic type shows 7 versus 11 in the winter, 7 versus 9 in spring, 8 versus 4 in summer, and 6 versus 7 in fall, respectively. CONCLUSION: There is no seasonal pattern to SOJRA disease onset in Israel. However, the disease onset of patients having the chronic or the polycyclic subtype tends to be more common in winter and spring. Since patients with this type have more severe disease, it is possible that another specific infectious agent is one of the factors involved in the pathogenesis of the disease. Larger sampling and multicenter studies are required to clarify this point.

Arthritis, Juvenile↗
PubMed1998

Autosomal recessive microcephaly with neonatal myoclonic seizures: clinical and MRI findings.

We describe an infant who was born to a consanguineous couple of Palestinian origin. The patient had severe microcephaly, myoclonic seizures, hypsarrythmia, spasticity, hypertonicity, and profound mental retardation. A similar case was reported in another unrelated Palestinian family, suggesting that this condition may be endemic. The condition resembles early onset myoclonic seizures and spasticity described by Tolmie et al.: Am J Med Genet 27:583-594 [1987]. To the best of our knowledge, only four pairs of sibs have been described with this syndrome; however, to date no magnetic resonance imaging (MRI) findings were reported for this condition. We present the clinical and radiological findings in the patients, including the first report of MRI findings.

Consanguinity↗
PubMed1998

Colchicine-induced myoneuropathy in childhood.

UNLABELLED: Colchicine is used in the treatment of gouty arthritis, familial Mediterranean fever, amyloidosis, Behcet disease and dermatoses. Myoneuropathy is a rare side-effect reported either with intoxication or in elderly patients with chronic renal insufficiency causing elevated plasma drug levels. We report the first two cases of myoneuropathy in children, both taking appropriate doses of colchicine, and having normal renal function. The myoneuropathic changes were reversible after stopping treatment. The cause of colchicine myoneuropathy is unclear. CONCLUSION: In children treated with colchicine, neuromuscular phenomena of unknown aetiology may be related to the drug, even with a lack of intoxication or renal insufficiency.

Adolescent↗
PubMed1998

Reversible cortical atrophy and cognitive decline induced by valproic acid.

An 18-year-old male suffered from familial progressive myoclonic epilepsy from the age of 7 years. In addition to seizures, there was a marked decline in school performance. At the age of 14 years, sodium valproate was started as add-on therapy; 2 weeks later he was hospitalized in a stuporous state. The serum level of valproate was within the therapeutic range. Cognitive evaluation disclosed moderate mental retardation. No metabolic abnormalities were detected. Valproate was discontinued and during the 4 following months, a slow but significant improvement was documented in cognitive functions. Repeated assessment was within the range of mild mental retardation. Initially, magnetic resonance imaging (MRI) showed mild cortical atrophy. A subsequent MRI study performed 2 years later was normal.

Adolescent↗
PubMed1997

Treatment of herpes simplex gingivostomatitis with aciclovir in children: a randomised double blind placebo controlled study.

OBJECTIVES: To examine the efficacy of aciclovir suspension for treating herpetic gingivostomatitis in young children. DESIGN: Randomised double blind placebo controlled study. SETTING: Day care unit of a tertiary paediatric hospital. SUBJECTS: 72 children aged 1-6 years with clinical manifestations of gingivostomatitis lasting less than 72 hours; 61 children with cultures positive for herpes simplex virus finished the study. MAIN OUTCOME MEASURES: Duration of oral lesions, fever, eating and drinking difficulties, and viral shedding. INTERVENTION: Aciclovir suspension 15 mg/kg five times a day for seven days, or placebo. RESULTS: Children receiving aciclovir had oral lesions for a shorter period than children receiving placebo (median 4 v 10 days (difference 6 days, 95% confidence interval 4.0 to 8.0)) and earlier disappearance of the following signs and symptoms: fever (1 v 3 days (2 days, 0.8 to 3.2)); extraoral lesions (lesions around the mouth but outside the oral cavity) (0 v 5.5 days (5.5 days, 1.3 to 4.7)); eating difficulties (4 v 7 days (3 days, 1.31 to 4.69)); and drinking difficulties (3 v 6 days (3 days, 1.1 to 4.9)). Viral shedding was significantly shorter in the group treated with aciclovir (1 v 5 days (4 days, 2.9 to 5.1)). CONCLUSIONS: Oral aciclovir treatment for herpetic gingivostomatitis, started within the first three days of onset, shortens the duration of all clinical manifestations and the infectivity of affected children. Further studies are needed to evaluate the ideal dose and length of treatment.

Acyclovir↗
PubMed1997

Fixed drug eruption of the penis due to hydroxyzine hydrochloride.

OBJECTIVE: To report four cases of fixed drug eruption induced by hydroxyzine hydrochloride (Otarex, Teva, Israel). CASE SUMMARY: Four children with restlessness who were treated with hydroxyzine hydrochloride developed fixed drug eruption of the penis. Drug discontinuation was followed by complete resolution of the skin eruption. Rechallenge resulted in the same drug rash. Macrophage migration inhibiting factor (MIF) assay with hydroxyzine hydrochloride was positive. DISCUSSION: The pathogenesis of fixed drug eruption and the role of macrophage MIF assay in diagnosis is discussed. CONCLUSIONS: A fixed drug eruption induced by hydroxyzine hydrochloride is possible, but is a rare phenomenon.

Animals↗
PubMed1997

Anti-Ro (SSA) and anti-La (SSB) antibodies and complete congenital heart block.

Complete congenital heart block (CCHB) is the most severe manifestation of neonatal lupus syndrome, associated with a mortality rate of up to 31%. It is caused by irreversible damage to the cardiac conduction system due to the transplacental passage of maternal antibodies to the fetus. Anti-Ro (SSA) and anti-La (SSB) antibodies are usually detected in sera of mothers of children with prenatally diagnosed CCHB and in cardiac tissues of affected newborns. Their pathogeneic role in the development of CCHB has been established in several studies. When CCHB is detected during pregnancy, careful monitoring and delivery are needed before heart failure is developed. Treatment strategies are debatable and may include prophylactic therapy for high-risk pregnant women and a combination of intrauterine plasmapheresis with plasma exchange or with corticosteroids. Pacemaker insertion is required in most infants during the first three months of life. Assays for anti-Ro (SSA) and anti-La (SSB) antibodies should be performed on sera of pregnant women with SLE and newborns with CCHB.

Antibodies↗
PubMed1996

Viscosity and refractive index of follicular fluid in relation to in vitro fertilization.

PURPOSE: To set the standard values of follicular fluid viscosity and refractive index, and to investigate a possible relationship between these physiological parameters and the outcome of in vitro fertilization treatment. DESIGN AND RESULTS: 128 samples of follicular fluid were collected from 40 in vitro fertilization patients. Viscosity determinations (centipoise; mean +/- SD) for shear rates of 23, 46, 115, and 230 were 2.04 +/- 0.86, 1.84 +/- 0.49, 1.48 +/- 0.27, and 1.38 +/- 0.22, respectively. The average (+/- SD) refractive index was 1.030 +/- 0.002. There was no significant difference between the values of thawed frozen fluids and fresh samples of the same specimens. The data showed no correlation between follicular fluid viscosity or refractive index and the presence of oocytes, their maturation grade or their fertilizing capacity. CONCLUSIONS: For the first time, values of the viscosity and refractive index of follicular fluid obtained during in vitro fertilization have been determined. However, these preliminary results did not reveal any relationship between the physiological parameters examined and the outcome of in vitro fertilization treatment.

Female↗
PubMed1996

A 'sweet' indication for ovulation induction.

In diabetic patients, euglycaemia at the time of conception is crucial for the success of the pregnancy. In consideration of the difficulty in achieving and maintaining tight glycaemic control for long periods, we administered clomiphene citrate, which is usually indicated in cases of absent or infrequent ovulation, to enhance fecundability in 10 pregestational diabetic patients. All conceived within one to three cycles of the drug, and nine delivered healthy term babies after uneventful pregnancies; one aborted spontaneously in the eighth gestational week. No effect of the drug on the diabetes was noted as based on measurements of glycosylated haemoglobin and fructosamine concentrations and the absence of changes in the patients' insulin requirements. In the light of these successful results, and in view of the importance of euglycaemia at the beginning of diabetic pregnancies, we suggest a new 'sweet' indication for the use of clomiphene citrate.

Adult↗