Biomedical subjects

M S Klein-Gitelman

Publications and source records attributed to M S Klein-Gitelman.

5 recordsLinked to original sources

PubMed2003

Lupus headaches in childhood-onset systemic lupus erythematosus: relationship to disease activity as measured by the systemic lupus erythematosus disease activity index (SLEDAI) and disease damage.

The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) is the most commonly used measure of disease activity for children with systemic lupus erythematosus (SLE). For headaches to be scored in the SLEDAI as a symptom of active disease, they have to be nonresponsive to narcotic analgesia. This may affect the overall estimation of disease activity, especially because headaches are common among children with SLE and narcotic analgesia is rarely used for headache therapy in paediatrics. Moreover, the importance of headaches for the development of damage and their relation to other clinical parameters and outcomes has not been well described for children with SLE. We reviewed the medical charts of an inception cohort of children (n = 63) who were newly diagnosed with SLE. Information on headaches and other disease parameters was obtained. Disease activity and damage were measured using the SLEDAI and the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index (SDI), respectively. It has been shown that the accumulated burden of active disease as measured by serial SLEDAI scores over time is one of the best predictors of eventual damage to children with SLE. New-onset or significant increase of severe and/or persistent headaches (LHA) were reported in 43% of the patients during a mean follow-up of 3.6 years. LHA occurred preferentially among patients with elevated levels of antiphospholipid antibodies (aPL) (P < 0.02) and only 6% of all LHA episodes were treated with narcotics and thus considered for the measurement of disease activity in the SLEDAI. LHA were unrelated to proxy-measures of disease activity, such as the need to intensify therapies. When used in children, the insensitivity of the SLEDAI to capture LHA did not seem to decrease the responsiveness of the SLEDAI to detect clinically important worsening of disease, or negatively impact on its ability to predict damage.

Adolescent↗

PubMed2000

The economic impact of intermittent high-dose intravenous versus oral corticosteroid treatment of juvenile dermatomyositis.

OBJECTIVE: To perform a cost-identification and cost-effectiveness analysis comparing oral corticosteroids (OCS) with high-dose intermittent intravenous corticosteroid (IVCS) regimens in the treatment of juvenile dermatomyositis (JDM). METHODS: Children previously diagnosed and treated for JDM (without myositis-specific or myositis-associated autoantibodies) at a single medical center by a single provider were identified. Two treatment protocols were compared: OCS and IVCS. Data on initial disease severity, time to remission, resource use, and costs generated were collected from patient records. Incremental cost-effectiveness ratios (ICE) were constructed. RESULTS: Patients treated with IVCS achieved median remission 2 years earlier at median increased cost of $13,736. The ICE ratio comparing IVCS to OCS is $6,868 per year of disease avoided. CONCLUSION: This study suggests that, although IVCS treatments are costly, they are cost-effective.

Administration, Oral↗

PubMed1998

Intravenous corticosteroids: adverse reactions are more variable than expected in children.

OBJECTIVE: To determine the frequency and severity of adverse reactions associated with high dose intermittent intravenous corticosteroids (IVCS) in children with rheumatic disease. METHODS: Prospective documentation of adverse reactions associated with IVCS given to 213 pediatric rheumatology patients over a 4 year period. RESULTS: Forty-six of the 213 children (22%) reported an adverse reaction. The 46 patients received 2622 doses of IVCS. Twenty-one patients (10% of all patients studied) had behavioral changes, including altered mood (14), hyperactivity (4), psychosis (2), disorientation (1), and sleep disturbances (3). Nonbehavioral adverse reactions included headache (5.2%), abdominal complaints (4.7%), pruritus (4.2%), vomiting (3.8%), hives (2.3%), hypertension (2.3%), bone pain (1.5%), dizziness (1.5%), fatigue (1%), lethargy (1%), hypotension (1%), tachycardia (1%), hyperglycemia (1%), fracture (1%), tremor (0.5%), anaphylaxis (0.5%), ulcer (0.5%), and "gray appearance" (0.5%). Using chi-squared analysis, there were no statistical differences in ethnicity (p = 0.54) or diagnosis (p = 0.46) between patient groups, with or without adverse reactions. There was a significant statistical association between history of drug induced cutaneous reaction and adverse reactions to IVCS (p < 0.01). CONCLUSION: IVCS are associated with a spectrum of adverse reactions in children with rheumatic disease, of which volatile behavior is the most frequent. Children with a history of drug induced cutaneous reaction are more likely to have an adverse reaction to IVCS.

Adolescent↗

PubMed1991

Disseminated Nocardia brasiliensis infection: an unusual complication of immunosuppressive treatment for childhood dermatomyositis.

The use of steroids combined with cytotoxic drugs has increased in the last decade. The concomitant increase of opportunistic infections has contributed significantly to morbidity and mortality of patients treated with immunosuppressive agents. We describe a child with dermatomyositis who developed disseminated Nocardia brasiliensis infection while receiving steroids and methotrexate. Infectious etiology was established by gram stain. The patient was treated successfully. Disseminated Nocardia brasiliensis infection is rare with a high reported mortality. Diagnosis may be delayed secondary to insidious onset, similarity of clinical manifestations to other pathogens and slow growth in routine culture media. Nocardia should be considered early in the evaluation of infection in patients treated with immunosuppressive agents.

Adolescent↗

PubMedDate not supplied

Systemic lupus erythematosus.

Systemic Lupus Erythematosus (SLE) of childhood is a complex and challenging disease which can occur at any age. Identification of disease early in it's course and aggressive, appropriate management leads to improved outcome for an individual child. The history of SLE indicates how much progress has been made in the last quarter century. A discussion of the etiopathogenesis of SLE demonstrates the complexity of the syndrome. This is followed by a description of clinical manifestations, including diagnostic criteria, differential diagnosis and suggested methods for eliciting important symptoms to make the diagnosis. Evaluation of specific organs is next reviewed highlighting critical organ manifestations that are significant for future prognosis. Treatment of SLE includes a variety of medications, including non-steroidal anti-inflammatory medications, steroids and immuno-suppressive drugs. Attention to physical activity, stress and nutrition is equally important. Signs and symptoms that indicate disease flare or infection are described. Lastly, related syndromes are reviewed.

Adrenal Cortex Hormones↗