Prevalence of Microalbuminuria in Adult Patients with Sickle Cell Disease in Eastern Saudi Arabia.

BACKGROUND: Proteinuria is a common feature of sickle cell nephropathy (SCN) that can progress to renal insufficiency and end stage renal disease. Microalbuminuria (MA) is the earliest manifestation of SCN and precedes the development of overt proteinuria. In addition to the renal consequences, MA is linked to cardiovascular complications. Periodic screening and early detection of MA allow early intervention that may reduce the risk of progression to advanced renal failure and cardiovascular diseases. OBJECTIVE: The aim of this study was to investigate the prevalence of MA in patients with SCD in the eastern region of Saudi Arabia. METHODS: A prospective cross-sectional observational study was conducted at Johns Hopkins Aramco Healthcare (JHAH). Urine samples of SCD patients 18 years old and older were tested for the presence of MA using urinary albumin over creatinine ratio (ACR). Correlation was tested with multiple variables including age, gender, body mass index (BMI), hemoglobin level, blood pressure, blood transfusion history, pain episodes, and use of hydroxyurea. RESULTS: Urine samples were tested on 72 patients. The mean age of the study cohort was 35 ± 16.9 years. Microalbuminuria was detected in 18 patients (25%). No correlation was found with any of the tested variables. CONCLUSION: Microalbuminuria is a common finding in patients with SCD in eastern Saudi Arabia. Patients with SCD should be screened for MA, and those with positive tests should probably be treated with antiproteinuric agents that may slow the progression to advanced stages of renal failure and decrease the risk of cardiovascular diseases.

End-stage renal disease in patients with sickle cell disease.

Sickle cell nephropathy is a severe complication of sickle cell disease (SCD) that has a wide range of manifestations, from asymptomatic microalbuminuria to end-stage renal disease (ESRD). The data on patients with SCD who develop ESRD are scarce. The aim of this study was to explore the course of patients with SCD who developed ESRD and received renal replacement therapy (RRT). The course of patients with SCD who developed ESRD and started dialysis at two centers in the Eastern Province of Saudi Arabia was retrospectively analyzed. Parameters included age at initiation of dialysis, survival until death or kidney transplantation, hospitalization due to pain crisis, disease-related parameters, and requirement for blood transfusion. Sixteen patients with SCD developed ESRD and started RRT with either hemodialysis or peritoneal dialysis. The mean age at initiation of dialysis was 46.6 years. The majority of patients (10 out of 16) were resistant to erythropoiesis-stimulating agents (ESA) and required blood transfusion repeatedly. Pain crises were infrequently encountered. Median survival was 54 months. Four patients received kidney transplantation with good outcome. In conclusion, most patients with SCD who developed ESRD were resistant to ESA and required repeated blood transfusion. The rate of hospitalization due to pain crisis was relatively low. Survival on dialysis was comparable to that of patients with no SCD, and the post-transplant course was relatively benign.

Proteinuria in patients with sickle cell disease.

Proteinuria is a complication of sickle cell nephropathy that can progress to renal insufficiency and end-stage renal disease. The magnitude of proteinuria among patients with sickle cell disease (SCD) has been reported with variable prevalence. The aim of this study was to determine the prevalence of proteinuria in a large number of patients with SCD in Eastern Saudi Arabia. The urinalyses of 940 non-diabetic patients with SCD were tested for the presence of proteinuria. The glomerular filtration rate (e-GFR) of all patients was estimated using the Cockcroft- Gault equation. Proteinuria was found in 79 of 940 patients with SCD (prevalence 8.4%). The mean age of the affected patients with proteinuria was 33 ± 15.4 years (10-76). The mean GFR was 118 mL/min/1.73 m 2 . This study indicates that patients with SCD in Eastern Saudi Arabia have a low prevalence of proteinuria and preserved GFR.

Listeria monocytogenes brain abscess in a patient with multiple myeloma.

Listeria monocytogenes is an uncommon cause of illness in the general population. Meningoencephalitis is the most common central nervous system (CNS) manifestation of listeriosis. However, brain abscess represents 1-10% of all CNS listeriosis. To our knowledge, L. monocytogenes brain abscess in multiple myeloma patients has not been previously reported. Thus we report a 58-year-old male patient with multiple myeloma who developed a brain abscess due to L. monocytogenes. Due to a history of penicillin allergy, he was treated with intravenous trimethoprim/sulfamoxazole (TMP-SMX) for a total of 12 weeks, and gentamicin for the first two weeks, followed by oral therapy of TMP-SMX for a total of nine months. He is alive six and a half years after the diagnosis of myeloma with occasional brief seizures despite being on two anticonvulsants.

Disseminated systemic Nocardia farcinica infection complicating alefacept and infliximab therapy in a patient with severe psoriasis.

Nocardiosis is a cause of significant morbidity and mortality in the immunocompromised host, and is an infrequent complication of tumor necrosis factor alpha (TNF-alpha) blockers in chronic inflammatory diseases. Nocardiosis occurs at a rate of 3.55 and 0.88 per 100 000 patients treated with infliximab or etanercept, respectively. Disseminated nocardiosis remains an uncommon complication of these agents. Here, we present a fatal case of disseminated systemic nocardiosis in a patient with psoriasis following sequential therapy with alefacept and then infliximab therapy. The patient developed disseminated disease involving the brain, lymph nodes, and adrenal glands. The diagnosis was made by blood culture and aspiration of the adrenal gland abscess, which revealed Gram-positive bacilli and later grew Nocardia farcinica. The organism was identified by DNA sequencing, and was susceptible to moxifloxacin, gatifloxacin, ciprofloxacin, amoxicillin-clavulanic acid, linezolid, sulfamethoxazole, and amikacin. It was resistant to clarithromycin, ceftriaxone, and tobramycin and was intermediately susceptible to imipenem.

Metachronus malignant rhabdoid tumor of the ileum and adenocarcinoma of lung: a unique case report.

Malignant extrarenal rhabdoid tumors had been described in a variety of anatomic locations including gastrointestinal tract. Carcinomas of small intestine are quite uncommon neoplasms, and those with rhabdoid differentiation are exceedingly rare. These poorly differentiated tumors pose a great deal of difficulty in diagnosis as well as in deciding whether they are primary or metastatic in origin because malignant rhabdoid tumors or carcinomas with rhabdoid differentiation of other sites can metastasize to the small intestine. They behave more aggressively than the typical adenocarcinomas of the same location. Herein, we report a 52-year-old patient with primary small bowel malignant rhabdoid tumor, who was completely disease-free after the initial presentation and management. Five years later, he was found to have a lung mass proved to be adenocarcinoma, exhibiting focal giant cell differentiation without rhabdoid features.

Are we underestimating the leukemogenic risk of hydroxyurea.

Hydroxyurea is an established drug that has been used for the treatment of myeloproliferative disorders and some solid tumors for some time. In recent years it has also been found to be effective in the treatment of sickle cell disease. Short term side effects are not serious, and are manageable. The major concern is the potential leukemogenesis with long term use. The risk of leukemogenesis is not defined with its use in benign hematological conditions. We report a case of acute myeloid leukemia with no preceding myelodysplastic syndrome, occurring after 2 years of hydroxyurea therapy in a patient with sickle cell disease.