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Biomedical subjects

R Straussberg

Publications and source records attributed to R Straussberg.

At least 37 records · Page 2Linked to original sources
PubMed2000

Successful treatment of rheumatic chorea with carbamazepine.

Carbamazepine has been used successfully in the treatment of different movement disorders and was recently reported to be effective for nonhereditary chorea. In view of the significant side effects associated with the drugs currently used to treat chorea, we sought to further evaluate the efficacy of carbamazepine in children with rheumatic chorea. The study was prospective and included 10 children with chorea (eight females and two males; age range = 7-16 years) referred to our Pediatric Rheumatology Clinic between 1995 and 1999. Nine had rheumatic fever and one had antiphospholipid antibody syndrome that later evolved to systemic lupus erythematosus. All were treated with carbamazepine. Improvement was evident within 2-14 days of initiation of low doses of carbamazepine (4-10 mg/kg daily). The plasma drug levels were 2.8-8.2 microg/mL (therapeutic antiepileptic range = 8-12 microg/mL). The chorea disappeared within 2-12 weeks. The duration of treatment was 1-15 months. No side effects were observed. Recurrence was observed in three patients who received a second trial of carbamazepine with a good response. We suggest that carbamazepine may serve as a first-line treatment for rheumatic chorea.

Adolescent↗
PubMed2000

Reversible deafness caused by biotinidase deficiency.

We present a child with complete biotinidase deficiency who developed bilateral sensorineural deafness without a response to a maximal stimulus of 90 dB in brainstem acoustic-evoked response. After treatment with 20 mg biotin daily, a repeated brainstem acoustic-evoked response demonstrated an improved hearing threshold of 65 dB, and the child began to talk. The case is a rare example of reversible hearing loss caused by to biotinidase deficiency and highlights the need for immediate replacement therapy once the diagnosis is established.

Amidohydrolases↗
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

Phototherapy for neonatal hyperbilirubinemia affects cytokine production by peripheral blood mononuclear cells.

The capacity of peripheral blood mononuclear cells (PBMC) to produce interleukin (IL) IL-1beta, IL-2, IL-3, IL-6, IL-10 and tumor necrosis factor-alpha (TNFalpha) was examined in term newborns with hyperbilirubinemia after 24 hours' exposure to phototherapy (wave length 425-475 nm). The results were compared with those from untreated neonates. Fifty newborns spontaneously delivered at term were included in the study. Blood samples were collected from 20 newborns before and 24 h after phototherapy. The control group consisted of 30 neonates examined on two consecutive days. PBMC isolated from blood samples were incubated in vitro for cytokine production. The concentration of cytokines in the supernatants was tested using ELISA kits (for IL-1beta, IL-6, IL-10 and TNFalpha), or by bioassays (for IL-2 and IL-3). Phototherapy caused a 70% increase in IL-2 secretion (123 +/- 27 vs 208 +/- 30 units/ml, P < 0.01) and 56% in IL-10 production (1.07 +/- 0.19 vs 1.67 +/- 0.33 ng/ml, P < 0. 03), whereas the spontaneous secretion of IL-1beta was reduced by 43% (13.7 +/- 2.3 vs 7.3 +/- 1.7 ng/ml, P < 0.02). In the control group the secretion of these cytokines was similar on the two consecutive days and did not differ significantly from secretion in the other group before phototherapy. On the other hand, lipopolysaccharide induced TNFalpha production was higher on the second day in the two groups of newborns irrespective of phototherapy (388 +/- 58 vs 683 +/- 88 pg/ml, P < 0.001, in the control group and 384 +/- 75 vs 588 +/- 91, P < 0.05, before and after phototherapy). The synthesis of IL-3 and IL-6 did not change significantly between the two days of the study. The results demonstrate that in addition to the well-known positive effect of phototherapy on the neonate serum bilirubin level, this treatment affects the function of the immune system in newborns via alterations in cytokine production.

Cytokines↗
PubMed1999

Effects of dexamethasone on IL-1beta, IL-6, and TNF-alpha production by mononuclear cells of newborns and adults.

The effects of dexamethasone on the production of interleukin (IL) 1beta, IL-6, and tumor necrosis factor alpha were studied in preterm newborns, term infants, and adults. Twenty preterm and 22 term newborns and 30 healthy adults were included in the study. Mononuclear cells (MC) isolated from cord blood of newborns and peripheral blood of adults were incubated without or with lipopolysaccharide in the absence or presence of dexamethasone at concentrations between 10(-8) and 10(-5) M. The cytokine concentration in the supernatants was tested using enzyme-linked immunosorbent assay kits. Although a dose-dependent inhibition of the cytokine production was observed at pharmacological doses of dexamethasone in individuals of the three groups, differences in the intensity of the effect were observed between the groups. Spontaneous secretion of IL-1beta or IL-6 by MC of preterm neonates was less inhibited by dexamethasone as compared with cells from adults. In contrast, the inhibitory effect of the drug on lipopolysaccharide-induced IL-6 and tumor necrosis factor alpha production was more pronounced on neonatal cells. As for term newborns, MC were more sensitive to the inhibitory effect of the drug on LPS-induced IL-6 production than cells of adults. The results suggest that dexamethasone treatment of preterm newborns may affect cytokine production with a consequent modulation of the host's immune response.

Adult↗
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↗
PubMed1998

3-Methyl glutaconic aciduria in Iraqi Jewish children may be misdiagnosed as cerebral palsy.

It is generally accepted that patients with cerebral palsy suffer from a static encephalopathy causing a non-progressive disorder of posture and/or movement. We describe 7 patients from 5 families who were initially diagnosed with cerebral palsy. Eventually, excessive excretion of urinary 3-methyl glutaconic acid (3-MGA) was found. The data of our 7 patients are quite similar to the clinical description of Costeff et al (1989): Jewish-Iraqi origin (7/7), consanguinity (2/7), involuntary movements (5/7), ataxia (6/7), pyramidal involvement (6/7) and optic atrophy (6/7). The cognitive functions were intact in 5/7 and 2/7 showed mild to moderate mental retardation. The mean delay in the definitive diagnosis was 9 years. Cerebral palsy-like symptoms accompanied by optic atrophy and extrapyramidal signs should call for extensive metabolic evaluation including the determination of urinary 3-MGA.

Adult↗
PubMed1998

Effect of three days starvation on the phagocytic activity of rat peritoneal macrophages.

To examine the effect of starvation on the phagocytic activity of rat peritoneal macrophages, 8 animals were kept for 3 days on water supply only. The cells showed an increased capacity for superoxide anion production and a marked decrease in their ability to engulf latex particles. The corticosterone level of the fasting animals was markedly increased, an observation suggesting that the alteration of macrophage function could be hormonally mediated. The findings in the present study indicate that the decreased phagocytic capacity of peritoneal macrophages following starvation may be one of the factors responsible for the increased susceptibility to infections in malnourished individuals.

Animals↗
PubMed1997

Ultrastructure of human colostral cells.

The ultrastructural architecture of colostral cells of mothers of pre- and full-term infants is described. The polymorphonuclears were engaged in vivid phagocytosis of fat droplets. Similar findings were observed on the macrophages. The lymphocytes appeared normal in size and ultrastructure. A small number of eosinophils and basophils were also detected. The number of colostral cells was higher in the colostrum of mothers of preterm newborns. The number of the cells in the colostrum in mothers of both groups decreased with advancement of lactation.

Animals↗
PubMed1997

Congenital intractable diarrhea of infancy in Iraqi Jews.

We report on five patients who presented with intractable diarrhea starting during the first days of life. The patients belonged to four families of Iraqi Jewish origin. Autosomal recessive inheritance is suggested by parental consanguinity in three families and recurrence in another sib in one family. The patients were all born after uneventful pregnancy and labor, with birth weight in the normal range. There were no dysmorphic features. Three patients were breast fed. Diarrhea started between the first and eighth day of life. Diarrhea was of the secretory type. No pathogen was cultured from the stool. Jejunal biopsies performed on all patients ranged from normal to severe partial villous atrophy. The patients received different drug regimens with no beneficial effect and all are dependent on TPN. These findings and the common ethnic origin of the patients suggest that these patients have the same syndrome of congenital intractable diarrhea. No similar cases are known in other ethnic groups in Israel, suggesting a possibility of high gene frequency among the Jews of Iraqi origin.

Adrenal Glands↗
PubMed1997

Effect of lipid emulsion on IL-2 production by mononuclear cells of newborn infants and adults.

The in vitro effect of a lipid emulsion (intralipid) on interleukin-2 (IL-2) production by cord blood mononuclear cells (CBMC) of preterm and term newborn infants was examined and compared to that of peripheral blood mononuclear cells (PBMC) of adults. Intralipid, added at concentrations accepted in clinical practice, caused a dose-dependent inhibition of IL-2 activity tested by bioassay. IL-2 levels, tested by radioimmunoassay (RIA), were found to be reduced only in supernatants derived from CBMC of term infants and not in those derived from MC of preterm infants or adults. The capacity of the IL-2 dependent cell line CTLL-2 to respond to IL-2 was abolished in the presence of intralipid, suggesting an interference with the binding of IL-2 to its receptor on these cells. It is conceivable that administration of intralipid to preterm infants may interfere with the binding of IL-2 to the specific receptors on their activated lymphocytes, with a possible subsequent suppression of their immune response.

Adult↗