Poor specific antibody response immunodeficiency (dysgammaglobulinemia) predates systemic lupus erythematosus.

Poor specific antibody response is a well-known primary immunodeficiency that is related to hypogammaglobulinemia or common variable immunodeficiency (CVID). The co-existence of CVID or hypogammaglobulinemia and systemic lupus erythematosus (SLE) has been rarely described. In all reported cases, the diagnosis of SLE antedates CVID. We report a 15-year-old Saudi girl who was diagnosed with poor specific antibody response at age 6 years in the form of poor or no antibody response and dysgammaglobulinemia. She developed SLE with musculoskeletal and hematological manifestations, positive antinuclear antibody and high anti-dsDNA nine years later. She was treated with rituximab with good response.

Leukemic synovitis as a presentation of myelomonocytic blast crisis of chronic myeloid leukemia.

We describe a patient with a 2-month history of right shoulder monoarthritis and fever as the presenting symptoms of a subsequent diagnosis of chronic myeloid leukemia in blast crisis. Imaging studies showed changes consistent with leukemic infiltration of the soft tissues around the right shoulder joint and the proximal humerus. Immunophenotypic and morphologic analysis of the large number of cells obtained from the synovial fluid confirmed the shoulder synovitis to be an extramedullary manifestation of myelomonocytic blast crisis of chronic myeloid leukemia. The patient was not a candidate for aggressive chemotherapy treatment because of her poor overall condition, and she had no compatible donor for allogenic bone marrow transplantation. Her painful arthropathy was refractory to standard pain management but she achieved excellent pain relief with palliative radiation therapy. We conclude that the involvement of extramedullary sites by chronic myeloid leukemia blast cells can predate hematological blast crisis in some of chronic myeloid leukemia cases. Also, painful leukemic synovitis can be managed by low dose radiotherapy in a candidate who is refractory to chemotherapy and other medical therapy.

Fine-needle aspiration biopsy in the management of thyroid nodules in children and adolescents.

We reviewed the utility of fine-needle aspiration biopsy (FNAB) in the management of thyroid nodules in 41 children and adolescents (35 girls; mean age, 13.3 years). Cytologic findings were benign in 30, malignant in 2, suspicious in 6, and insufficient in 3. The malignancy rate was 5%, with no false-negative results. Diagnostic accuracy for FNAB was 87% and inter-observer reliability was 88%. FNAB is safe and highly accurate in the evaluation of thyroid nodules in childhood.

Deep vein thrombosis at King Abdul Aziz University Hospital.

OBJECTIVE: To study the incidence of deep vein thrombosis and pulmonary embolism at King Abdul Aziz University Hospital. To determine the risk factors, use of different diagnostic modalities, treatment given and to compare our findings with those reported in the literature. METHODS: Retrospective study which included all cases of deep vein thrombosis and pulmonary embolism admitted to the medical ward of King Abdul Aziz University Hospital during the period between January 1994 till March 1999 were analyzed. RESULTS: Total of 75 patients were diagnosed to have deep vein thrombosis with mean age of 44.16 +/- 14.5 years and male:female ratio of 1:2. Doppler ultrasound was used for the diagnosis in 56 of 75 patients (75%). Pulmonary embolism as a complication of deep vein thrombosis developed in 24 of 75 patients (32%). Prolonged immobilization was found to be the most common risk factor 17 of 75 (23%). All the patients were treated with conventional heparin followed by warfarin. CONCLUSION: As discussed, our results are comparable with those reported in the literature. Post operative patients who are anticipated to have prolonged immobilization should receive prophylactic anti coagulation with subcutaneous heparin. Thrombophillia screening should be reserved for those with recurrent deep vein thrombosis or patients with positive family history.

A survey of childhood hearing impairment.

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Renal involvement in Behçet's disease: review of 9 cases.

We describe clinical data on 9 patients with Behçet's disease with renal involvement (8 males, one female) and results of kidney biopsies in 4 patients. Renal involvement occurred in 9 of 120 patients (7.5%) followed at our center. Proteinuria was present in 8 patients (6 with quantitative 24 h urine protein with a range of 0.32-3.11 g/day, mean 1.7 g/day); hematuria occurred in one patient. Impaired renal function was encountered in 2 patients at presentation and in another patient at a later date. Major vascular involvement (major venous and/or arterial thrombosis) was present in 4 patients and sagittal sinus thrombosis in another patient. Measurement of complement C3, C4, and CH50 and immunologic tests for anticardiolipin, antineutrophil cytoplasmic, and antistreptococcal antibodies did not indicate a clear association with renal involvement. Kidney biopsies were performed in 4 patients. Mesangial proliferative changes were observed in 3 patients and evidence of immune complex deposition by immunofluorescence and electron microscopic examination was present in 2 patients. Amyloidosis was present in one patient who had vascular involvement and presented with nephrotic range proteinuria.

Underlay tympanoplasty with anterior and posterior flaps for subtotal perforations.

In conventional underlay tympanoplasty, post meatal flap is elevated and graft placed medial to the handle of maleus. Out of this present series of 200 ears, in 90 cases posterior flap technique and in 110 cases both posterior and anterior flap was applied and results are studied after median observation time of 20 months. The success rate with combined anterior/posterior flap is better compared to the posterior flap alone. Different techniques described by various authors for tympanoplasty are also discussed in this paper.

Reversible uremic deafness: is it correlated with the degree of anemia?

Hearing loss is a common finding in patients with end-stage renal failure. Uremic toxins, ototoxins, and axonal uremic neuropathy appear to be likely pathogenic factors. We analyzed whether an improvement in hearing capacity can be achieved with an improvement of anemia by erythropoietin (EPO) administration. Fifty patients on long-term hemodialysis in a single center were examined audiologically by otoscopy, tympanometry, pure tone audiometry, and the short increment sensitivity index. Twenty-five patients were treated with EPO in a dose of 120 U/kg per week over a period of 5 to 8 months, and the remaining 25 patients were not treated with EPO (controls). Both groups were reexamined audiologically after the study period, and the results were compared. In the group treated with EPO, the hemoglobin level increased from 7 +/- 0.9 to 11 +/- 0.8 g/dL, as against the control group, whose hemoglobin increased from 7.1 +/- 0.9 to 8 +/- g/dL. The audiologic tests were repeated at the end of the study period, and a significant improvement of hearing was found in the patients treated with EPO as compared with the control group (p < .001). Our study suggests that improvement of anemia in patients on long-term hemodialysis by administration of EPO is associated with an improvement in hearing capacity in a significant number of patients. Thus, anemia seems to be an important factor responsible for hearing disorders in patients with end-stage renal failure. Studies with larger numbers of patients are required to confirm this observation.

Use of magnetic resonance imaging in diagnosing eosinophilic fasciitis. Report of two cases.

OBJECTIVE: To determine the role of magnetic resonance imaging (MRI) in the diagnosis of eosinophilic fasciitis (EF), selection of appropriate biopsy site, and followup of treatment. METHODS: MRI was used to examine 2 patients with EF at the time of their initial clinical presentation and after several months of treatment. T2-weighted axial, T2-weighted axial with fat saturation, and T1-weighted axial post-gadolinium with fat saturation scans at 1.5T were obtained. RESULTS: MRI demonstrated hyperintensity within the fascia. This defect resolved with treatment and clinical improvement. CONCLUSION: MRI is a useful noninvasive tool for diagnosing EF and for monitoring the effectiveness of therapy.

Pattern of adult onset of polymyositis and dermatomyositis and association with malignancy.

A retrospective study of 22 adult patients with dermatomyositis (DM) or polymyositis (PM) was performed. Male to female ratio was 1:2.7. Mean age of onset was 37.3 +/- (16.3) and symptoms were present for a mean of 11.2 +/- 14.6 months before diagnosis. Primary polymyositis was diagnosed in 11 (50%), primary dermatomyositis in three (13.6%). PM/DM was associated with connective tissue disease in three (13.6%) and malignancy in five patients (22.7%). Muscle disease followed the diagnosis of malignancy by a mean of 12.2 months (one to 36 months). All were female. Diffuse erythema was observed in all three patients with DM and malignancy. Arthritis was seen more frequency in our patients (55%). Sixty-eight percent of patients showed substantial improvement of muscle disease with steroids alone or in combination with other immunosuppressive agents, 18% did not improve or their disease progressed in spite of the treatment. Three patients died (14%), two from respiratory failure and one from underlying malignancy.