how to cite this article: mansouri nejad se, yazdan panah mj, tayyebi meibodi n, ashrafzadeh f, akhondian j, beiraghi toosi m, eslamieh h. griscelli syndrome: a case report. iran j child neurol. 2014 autumn;8(4): 72-75. objective griscelli syndrome (gs) is a rare autosomal recessive immune deficiency disorder that presents with pigmentary dilution of the skin and hair, recurrent skin and pulmon...

patients with organic acidemia are prone to different infections, which lead to acidosis episodes. some studies have evaluated the status of immune system in acidotic phase in these patients, but to the best of our knowledge no study has evaluated the immune system in non-acidotic phase of the disease. in this study, thirty-one patients with organic acidemia were enrolled. for evaluation of hum...

background: primary immunodeficiency disorders (pid) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. objective: to evaluate the demographic and clinical data of pid patients diagnosed in a referral pediatric hospital. method: all pid cases with a confirmed diagnosis,...

One of the neurologic complications of human immunodeficiency virus infection are cerebrovascular accidents. In HIV infected patients, ischemic strokes have been reported secondary to nonbacterial thrombotic endocarditis and cerebral arteritis. We describe an unusual cause of stroke in HIV-1 infection: Herpes Zoster ophtalmicus with contralateral hemiplegia.

Diagnosis and treatment of syphilis are challenging because of the condition's diverse clinical symptoms, histopathological variance, and the lack of definite tests for treatment follow-up. We report a case of secondary pustular-ulcerative malignant syphilis with ocular involvement in a human immunodeficiency virus-infected patient. It was striking to find that ulcerative lesions can be highly ...

We report on a human immunodeficiency virus-infected man undergoing urgent anorectal surgery, with multi-centimeter fungating masses discovered inside the anus. Initial pathology was inconclusive. After the patient developed a disseminated rash postoperatively determined to be secondary syphilis, the anorectal pathology was reviewed and Treponema pallidum DNA was amplified by polymerase chain r...

We report a case of retinal toxoplasmosis that occurred in a patient with acquired immunodeficiency syndrome who had a previous diagnosis of cerebral toxoplasmosis, despite the patient having had a robust immune response to highly active antiretroviral therapy. Clinical decisions about whether to discontinue secondary prophylaxis for opportunistic infections continue to be challenging.

Early diagnosis of severe combined immunodeficiency (SCID) is important to enable prompt referral to a supraregional centre for bone marrow transplantation before the occurrence of end organ damage secondary to infective complications. This review outlines clinical, microbiological, and immunopathological clues that aid the diagnosis of SCID and emphasises the multidisciplinary approach needed ...

Figure 1. A, CT scan before empirical therapy for toxoplasmic encephalitis in an HIV-infected patient. B, CT scan 2.5 years later, after immune restoration with highly active antiretroviral therapy and 8 months without maintenance therapy for toxoplasmic encephalitis. Discontinuation of Secondary Prophylaxis for Toxoplasmic Encephalitis in Human Immunodeficiency Virus Infection After Immune Res...

We describe a patient who presented with cryptococcosis and the adult respiratory distress syndrome (ARDS) as the initial manifestation of the acquired immunodeficiency syndrome. This patient represents the first reported recovery from ARDS secondary to widespread cryptococcosis. He is currently doing well as an outpatient on maintenance therapy with amphotericin B and azidothymidine.