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Biomedical subjects

S M Al-Mayouf

Publications and source records attributed to S M Al-Mayouf.

13 recordsLinked to original sources
PubMed2006

Juvenile systemic lupus erythematosus in multicase families from Saudi Arabia: comparison of clinical and laboratory variables with sporadic cases.

The object of this study was to compare patients with familial versus sporadic systemic lupus erythematosus (SLE) with respect to clinical, laboratory variables and outcome. The familial SLE group comprised 12 patients while the comparative group comprised 24 patients selected by systemic sampling from our pediatric rheumatology clinic database. Those patients are listed according to the date of referral, which represents a sampling frame. The first patient was chosen randomly and subsequent patients were chosen at intervals of three. The two groups were compared with respect to: demographic information, age of onset of SLE, disease and follow up duration, clinical and laboratory variables and outcome. The patients from the familial group were younger and had an earlier age of onset of disease (P = 0.03, 0.001 respectively). Seven patients with familial SLE were from the eastern region of Saudi Arabia (P = 0.006). The two groups were comparable with respect to gender, disease duration and follow-up. At diagnosis, the discoid rash was more frequent in the familial group (P = 0.03) while other clinical and laboratory variables including disease activity as measured by SLEDAI did not show significant differences. The mean dose of steroid and use of other immunosuppressive therapy were similar in both groups. Three patients from the familial group died; two of them had unusual complications (one patient had transverse myelitis and pancreatic pseudocyst and the other one had extensive pyoderma gangrenosum). All patients from the sporadic group are alive in stable condition but one patient had severe central nervous system disease. Familial SLE patients tend to be younger and more likely to have discoid rash, in addition a marked difference in the origin of patients was noted. These differences may be helpful in identifying SLE patients with a stronger genetic predisposition. The mortality among familial SLE patients is more frequent which may reflect the disease severity.

Adolescent↗
PubMed2002

Pancreatic pseudocyst in paediatric systemic lupus erythematosus.

Pancreatitis is a rare complication of paediatric systemic lupus erythematosus (SLE). We describe a child with severe form of SLE who initially developed acute pancreatitis, subsequently complicated by extensive pancreatic pseudocyst. The treatment and outcome are discussed.

Adolescent↗
PubMed2001

Cogan's syndrome in childhood.

We present a case of Cogan's syndrome in childhood presenting with arthralgia, myalgia, anorexia, uveitis, aortic regurgitation, and intermittent fever and who responded well to corticosteroid therapy and successful valvular replacement. The extensive diagnostic methods and a review of the literature are covered and intended to familiarize pediatricians with this rare but treatable disorder.

Administration, Oral↗
PubMed2001

Evaluation of parental knowledge of Pediatric Rheumatic Diseases.

OBJECTIVE: To investigate the parental knowledge of pediatric rheumatic diseases in general, and in particular information regarding their children's diseases. To focus on the important role of health education in understanding these chronic diseases, and formulate a future plan for establishing a general public education program. METHODS: One hundred sets of parents, of children with established rheumatic diseases with mean duration of illness, (4.1 +/- 2.83), a mean child age (9.9 +/- 3.15) years, were given a 20 multiple choice questionnaire during their routine visit to the Pediatric Rheumatology Clinic and Physiotherapy Department or to the pediatric ward at King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, over a 6-month period between December 1998 and June 1999. The questionnaire addressed 4 main areas: 1. parental awareness regarding their children's diagnosis and duration, 2. source of information and parental satisfaction, 3. general knowledge about rheumatic diseases and 4. knowledge about medical and physical therapy. The total possible score is 23. RESULTS: The overall mean score is 11.6. The mean total score is not affected by the parental education level. The majority of parents have wrong beliefs, regarding rheumatic diseases. The treating physician is the main source of parental information and in the majority of the parents, this information is satisfactory. CONCLUSION: The questionnaire is a simple and easy test to investigate parental knowledge regarding pediatric rheumatic diseases. The survey shows the need for health education programs and a future general public health education plan to improve awareness of pediatric rheumatic diseases and maybe other chronic illness.

Adolescent↗
PubMed2000

New form of idiopathic osteolysis: nodulosis, arthropathy and osteolysis (NAO) syndrome.

We describe 10 patients (6 females and 4 males) from 6 unrelated families with an autosomal recessive disease characterized by simultaneous presentation of nodulosis, arthropathy and osteolysis. They were followed up regularly at King Faisal Specialist Hospital and Research Center in Saudi Arabia for clinical evaluation, serial blood work-up, and evaluating radiological changes. Nodulosis and arthropathy were the clinical criteria for inclusion in this study, and the ten patients fulfilled these criteria. All patients had nodulosis and distal arthropathy. Eight patients (80%) presented with deformed hands and four (40%) with painful hands. All patients had parents who were first cousins and three families had more than one affected child, the finding suggesting autosomal recessive inheritance. Osteopenia and undertubulation of bones distally more than proximally, and upper limbs affected more often than lower limbs, were found in all patients. Osteolysis was seen in carpal and tarsal bones. Other common findings were sclerotic cranial sutures, brachycephaly, and broad medial clavicles. This novel phenotype should be considered in the differential diagnosis of chronic arthritis. Familial arthropathies are more often seen in communities where interfamilial marriage is common. Such a collection of patients is ideal for homozygosity mapping of the disease locus.

Adult↗
PubMed2000

Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety.

OBJECTIVE: To assess the efficacy of intravenous immunoglobulin (IVIG) for the treatment of juvenile dermatomyositis (JDM) in patients who were unresponsive to corticosteroids (steroid resistant or steroid dependent) or showed unacceptable toxicity. METHODS: A retrospective chart review of the course of all patients with JDM treated with IVIG who attended the Dermatomyositis Clinic at The Hospital for Sick Children, Toronto, Canada, from August 1986 to December 1996. RESULTS: Eighteen patients with JDM were treated with IVIG. Ten patients were taking additional 2nd line treatments, methotrexate, azathioprine, cyclosporine, and cyclophosphamide. The main indication for starting IVIG was the failure of steroid therapy to induce remission of JDM. Twelve patients showed clinical improvement with IVIG. In these patients, the corticosteroid dose was reduced by > 50% for > 3 months without clinical or biochemical flare. Nine of these 12 patients had IVIG alone as a 2nd line agent, whereas 3 patients were treated with additional agents. Six patients remained steroid dependent; they subsequently required multiple agents to induce remission of JDM. CONCLUSION: Most steroid dependent or steroid resistant patients in our clinic were able to markedly reduce their dose of corticosteroid with the addition of IVIG. Given the retrospective nature of our data and the fact that multiple agents were sometimes used together, it will be important to confirm these findings in a controlled trial.

Antirheumatic Agents↗
PubMedDate not supplied

Infections associated with juvenile systemic lupus erythematosus.

OBJECTIVE: To determine the rate and nature of infection in a cohort of children with SLE. METHODS: Records of 70 children with SLE were reviewed for documentation of infections. All patients with infection seen between 1990 and 1998 were included. Data extracted comprised demographics and clinical features including the disease activity index (SLEDAI), detailed descriptions of therapy, and response to antibiotic therapy. Infections were identified and catagorized as class I (minor) or class II (major). RESULTS: A total of 29 patients (41%) had infections; 73% had class I and 27% had class II infections. The most common cause of class II infection was bacteremia (45%), while urinary tract infection was more frequent (38%) in class I infections. There was no association between the severity of infection and various potential risk factors. CONCLUSION: Our data confirm that infection is common among children with SLE. There were no deaths directly related to infection.

Adolescent↗
PubMedDate not supplied

Infantile systemic hyalinosis: a fatal disorder commonly diagnosed among Arabs.

We retrospectively reviewed 19 patients (11 male, 8 female) with infantile systemic hyalinosis (ISH) seen at a tertiary care hospital. Fifteen patients (83.3%) presented in the neonatal period. The referral diagnosis was inaccurate in 14 patients (73.7%). Thirteen patients were products of first-degree cousin marriages (68%) and 5 families had more than one affected child. All patients had painful joint contractures and typical mucocutaneous changes (hyper-pigmented sclerodermatous skin over the knuckles and malleoli, gingival hyperplasia, subcutaneous and perianal fleshy nodules). Growth failure was noted in all of them and 39% had profuse diarrhea, 72% had low serum albumin. Radiological findings included osteopenia, periosteal reaction and osteolytic lesions. Tissue biopsy was consistent with the diagnosis in the 8 patients who had the biopsies. Despite aggressive management with physiotherapy and different medications (including NSAIDs, penicillamine and methotrexate), the disorder was progressive and none of them showed improvement. 16 patients died with a mean age of 11 months and only 3 are alive with a mean age of 20 months. This report represents the largest series of ISH. Our data suggests that ISH is a commonly diagnosed disorder in Saudi Arabia and among Arabs.

Arabs↗