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Biomedical subjects

J Amir

Publications and source records attributed to J Amir.

At least 37 records · Page 2Linked to original sources

Lactic dehydrogenase isoenzymes in cerebrospinal fluid associated with hydrocephalus.

UNLABELLED: Various neurological disorders are associated with specific changes in the level of total lactic dehydrogenase and concentrations of its isoenzymes in the cerebrospinal fluid. We describe the lactic dehydrogenase isoenzyme values in children with hydrocephalus. Cerebrospinal fluid samples collected from 10 patients (2 to 16 mo) with hydrocephalus were analysed for total lactic dehydrogenase activity and lactic dehydrogenase isoenzymes. Findings were compared with those in samples from 15 paediatric patients, with normal results. Mean total lactic dehydrogenase activity in the cerebrospinal fluid was significantly higher in the patients with hydrocephalus (101 +/- 23.11 U/L) than in the controls (33.53 +/- 5.75 U/L) (p <0.001). In the control samples, lactic dehydrogenase-1 was the main fraction, followed by lactic dehydrogenase-2 and 3; only small concentrations of lactic dehydrogenase-4 and lactic dehydrogenase-5 were detected. By contrast, patients with hydrocephalus had lower concentrations of the lactic dehydrogenase-1 fraction and higher lactic dehydrogenase-2 and lactic dehydrogenase-3 concentrations, the differences between these results and those in the control group being statistically significant (p < 0.001). The values for lactic dehydrogenase-4 and lactic dehydrogenase-5 were similar in both groups. CONCLUSION: Findings should be considered together with computed tomography/magnetic resonance imaging and ultrasound scans. The cerebrospinal fluid lactic dehydrogenase profile may prove to be an important predictor of cerebral injury, obstructive hydrocephalus and long-term neurodevelopmental problems.

Case-Control Studies↗

Early responses to nonconjugated polyribosylribitol phosphate challenge as evidence of immune memory after combined diphtheria-tetanus-pertussis-polio-Haemophilus influenzae type b primary vaccination.

OBJECTIVES: A high risk of invasive Haemophilus influenzae type b (Hib) disease exists in the first few years of life. A reduction in anti-polyribosylribitol phosphate (PRP) antibody concentrations follows the administration of certain diphtheria-tetanus-acellular pertussis (DTPa)-based Hib conjugate combined vaccines. However, these combined vaccines prime the immune memory, which is an important factor in protection. As yet there is no direct evidence of the time scale involved in the development of the immune memory post-primary vaccination. In this report we investigated the presence of immune memory at 10 and 12 months of age, 4 and 6 months after primary vaccination of young infants with a pentavalent combination of DTPa, inactivated poliovirus vaccine (IPV) and Hib (DTPa-IPV/Hib) vaccine. METHODS: In two trials (A and B) infants received DTPa-IPV combined with Hib-tetanus conjugate (PRP-T) vaccine at 2, 4 and 6 months of age. The presence of immune memory was assessed by measuring anti-PRP concentrations 7 to 10 days after a nonconjugated PRP challenge given at 10 months in Trial A and at 12 months in Trial B. RESULTS: Administration of a nonconjugated PRP challenge 4 and 6 months after primary vaccination in Trials A and B, respectively, elicited an increase in anti-PRP geometric mean concentrations (4.5 and 5.8 microg/ml, respectively) within 7 to 10 days. These concentrations exceed those reported in the literature involving unprimed children who had received a single dose of nonconjugated PRP at the same age. CONCLUSION: The results demonstrate the development of anti-PRP immune memory at an early age, 4 and 6 months after completion of a three dose primary vaccination course of combined DTPa-IPV/Hib vaccine. The ability of primed infants to mount a rapid response is an important observation given the high risk of Hib infection at this critical age.

Antibodies, Bacterial↗

Radial osteomyelitis as a complication of venous cannulation.

Venepuncture of the superficial veins in the forearm is considered a relatively safe procedure. We report two patients who presented with osteomyelitis of the proximal radius following venous cannulation of the median cubital vein, and one patient who developed osteomyelitis of the distal radius after cannulation of the cephalic vein. Osteomyelitis developing in proximity to a venepuncture site should raise the suspicion that a pathogen causing superficial thrombophlebitis has spread through the deep veins of the arm into the adjacent bone, thus causing osteomyelitis.

Adolescent↗

A syndrome of transient encephalopathy associated with adenovirus infection.

BACKGROUND AND OBJECTIVE: Adenovirus is a common pathogen in the pediatric population. Respiratory, gastrointestinal, or renal systems are often involved in adenovirus infections. Several neurologic syndromes have been attributed to adenovirus, such as adenovirus aseptic meningitis, myelitis, subacute focal encephalitis, and Reye-like syndrome. The purpose of this study was to describe the clinical features and encephalography findings in 7 infants treated in our center for a syndrome of transient encephalopathy associated with adenovirus infection. STUDY PARTICIPANTS: Three females and 4 males ages 7 to 34 months seen in our department between July 1983 and February 1984 and September 1998 and May 1999 presented with fever of at least 7 days' duration and a gradual decline in the state of alertness. Score on the Glasgow Coma Scale ranged from 9 to 12. Findings on lumbar puncture were normal. In all 7 patients, the encephalogram showed moderate to severe background slowing compatible with encephalopathy. All patients were catarrheal and had mild hepatomegaly with slight elevation of liver enzymes. Some had bronchopneumonia, diarrhea, and conjunctivitis either isolated or in combination. METHODS AND RESULTS: Adenovirus was isolated by immunfluorescence technique in all patients-from the sputum in 3 patients, nasopharynx in 5, conjunctiva in 4, and rectal swab in 5. In 5 patients, serotyping was performed by an antibody neutralization method. Adenovirus type 3 was ascertained from a nasal swab in 1 patient, sputum specimens in 3, throat swab in 3, and rectal cultures in 5. The clinical course was characterized by a progressive recovery of alertness. After several days, there was a complete reversal of neurologic findings. CONCLUSION: We suggest that this syndrome of transient encephalopathy is a distinct entity and should be considered as another of the several neurologic syndromes known to be associated with adenovirus infection.

Adenoviridae↗

Clinical aspects and antiviral therapy in primary herpetic gingivostomatitis.

Primary symptomatic herpes simplex virus infection in children usually manifests as gingivostomatitis and is prevalent in the 1- to 3-year age group. The disease involves the buccal and gingival mucosa and the tongue, and lasts approximately 2 weeks. Two recent non-blind studies reported a more rapid regression of symptoms after treatment with aciclovir suspension. Three randomised double-blind, placebo-controlled trials have clearly demonstrated that early aciclovir treatment significantly shortens the duration of all clinical manifestations and infectivity of affected children compared with placebo. We conclude that the treatment of herpetic gingivostomatitis with aciclovir is recommended. Treatment should be started within the first 3 days of disease onset. The proposed therapeutic dose is 15 mg/kg, 5 times daily for 5 to 7 days.

Acyclovir↗

Treatment of calcinosis in juvenile dermatomyositis with probenecid: the role of phosphorus metabolism in the development of calcifications.

OBJECTIVE: To report the efficacy of probenecid for calcinosis of juvenile dermatomyositis (JDM) and assess the changes in phosphorus metabolism during treatment. METHODS: Biochemical studies of calcium and phosphorus metabolism were performed in a 9-year-old girl with JDM and extensive calcifications before and during probenecid treatment. RESULTS: The calcifications resolved over 18 months of treatment. Probenecid was found to be effective in reducing calcifications by increasing renal phosphate clearance. CONCLUSIONS: The tendency for calcifications in some patients with JDM might be related to an increase in renal phosphate reclamation, and therefore, probenecid treatment may be effective in these patients.

Calcinosis↗

[Evaluation of radiation dose and radiation risk for dental patients].

1. The collective dental radiation dose in Israel in 1998 is 42.86 Sv. 2. The collective dental radiation is responsible for a mortality of about 2.14 people per year in Israel, and a similar number of morbidity from non-lethal cancer. 3. It seems that the quality of the picture in dental radiographs in Israel is not satisfactory, so is the dentists' level of knowledge about it. 4. Exposure to dental radiographs may be reduced by half if measures are taken to ensure the quality of the radiographs and the knowledge of dentists on the subject.

Clinical Competence↗

[Liver failure with coagulopathy in an infant with tyrosinemia].

Tyrosinemia is an inherited autosomal recessive condition. We present a 5 week-old boy with this disorder. He was admitted because of a fever, vomiting and lethargy. The laboratory tests confirmed a coagulopathy with prolonged prothrombin time (PT), partial thromboplastin time (PTT) and a decreased serum fibrinogen. The alpha-fetoprotein level was markedly elevated. To confirm the diagnosis of tyrosinemia, quantitative urinary succinylacetone was measured. Although overt liver failure with coagulopathy may be part of the representation of tyrosinemia, a significant coagulopathy in the absence of overt signs of liver disease has not been emphasized as a clue to the diagnosis of this condition.

Biomarkers↗

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↗

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↗

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↗

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↗

Immunization against hepatitis A in the first year of life: priming despite the presence of maternal antibody.

BACKGROUND: Maternal antibodies interfere with hepatitis A vaccination in young infants. We examined the response to a high dose hepatitis A vaccine administered concomitantly with a combination of diphtheria-tetanus toxoids-acellular pertussis-inactivated poliovirus vaccine/Haemophilus influenzae type b vaccine to initially seropositive vs. initially seronegative infants. METHODS: Three hundred subjects were originally planned to be enrolled at age 6 to 10 weeks and received hepatitis A vaccine (formalin-inactivated vaccine, SB-Bio, 720 enzyme-linked immunosorbent assay units) at 2, 4 and 6 months concomitantly with a diphtheria-tetanus toxoids-acellular pertussis-inactivated poliovirus vaccine/H. influenzae type b vaccine. Children initially seropositive received a booster dose at 12 months of age. An additional 100 twelve-month-old infants previously not vaccinated with hepatitis A vaccine were given 1 dose, to observe the primary response at that age. Reactogenicity was recorded on diary cards for the 3 subsequent days. Immunogenicity was measured at Months 2, 4, 5, 10 and 11 after administration of the first vaccine dose. For the subjects enrolled at 12 months, blood was drawn before and 1 month after the first vaccination. RESULTS: Of 297 initially enrolled infants 36% were seronegative before vaccination (Group A). The geometric mean concentration (GMC) (milli-International Units/ml) of the seropositive infants (Group B) before immunization was 2587. The GMCs of Group A infants 1 month after each dose and at 12 months of age were 93, 518, 1656 and 786, respectively. For Group B infants, the respective GMCs were 1165, 460, 508 and 167. One hundred subjects of Group B received a booster dose at age 12 months; at Month 13 all were seropositive with a GMC of 1902. For comparison, a third group of 100 not previously immunized 12-month-old infants (Group C) were enrolled and received 1 dose of hepatitis A vaccine with pre- and postimmunization GMCs of 52 and 120, respectively. CONCLUSIONS: Our results suggest that the initially seropositive infants were primed despite maternal antibody interference. The hepatitis A vaccine was well-tolerated in this population of young infants.

Female↗

Secondary enuresis: post-traumatic stress disorder in children after car accidents.

BACKGROUND: In our experience, secondary enuresis nocturna is a common complaint among children after a motor vehicle accident. However, as these children are often brought for examination as part of an insurance compensation claim, this complaint is not always reliable. OBJECTIVE: To describe a series of children in whom secondary enuresis occurred after a motor vehicle accident. METHODS AND RESULTS: Five children were brought to our clinic for evaluation of secondary nocturnal enuresis. Review of past history revealed a car accident preceding the onset of the enuresis. All but one had additional behavioral symptoms typical of post-traumatic stress disorder. Four children had evidence of head trauma, and one had psychological but no physical trauma. CONCLUSIONS: Nocturnal enuresis can occur after a motor vehicle accident due either to purely psychological trauma or organic head trauma. While nocturnal enuresis is generally attributed to organic causes, psychological mechanisms also play a significant role.

Accidents, Traffic↗

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↗