Hereditary spherocytosis with leg ulcers healing after splenectomy.

Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity. A refractory chronic leg ulcer is an uncommon complication of HS, reported in fewer than two percent of patients. We present the case of a 28-year-old man who was suffering from a leg ulcer and was unresponsive to treatment of two years' duration with various conservative modalities. He had been suffering from repeated attacks of jaundice, pallor, and splenomegaly since he was 10 years old. Upon admission, physical and laboratory findings revealed moderate HS. However, complete clearance of the leg ulcer was not achieved until two months after a splenectomy.

Diffuse uremic tumoral calcinosis in a patient on long-term hemodialysis.

Tumoral calcinosis is an uncommon and severe complication of hemodialysis therapy. The most important pathogenic factor involved in uremic tumoral calcinosis is an increase in calcium-phosphorus product, not necessarily related to hyperparathyroidism. We report here a patient on hemodialysis who presented with increasing multifocal and uncommon sites of massive calcifications. The patient was examined, and a diagnosis of uremic tumor calcinosis was made. The patient was treated with the noncalcemic phosphate binder sevelamer, a strict diet, multiple hemodialysis sessions per week, and a low calcium dialysate, with improvement on biological findings a decrease in the volume of some tumors on his fingers and a global stable disease. Some nodes in hands and feet disappeared; in other sites, their diameter was reduced, and the largest nodule decreased from 5- to 2-cm diameter.

[Nasal NK/T-cell lymphoma: about two rare cases]. / Lymphome T/NK nasal: à propos de deux cas rares.

NK/T-cell lymphoma is a severe and rare disorder in Africa and in Europe. Treatment is based on radiotherapy and chemotherapy. We here report two cases of elderly patients aged 55 years and 52 years, respectively, with bilateral nasal obstruction associated with purulent rhinorrhea. Clinical examination showed cleft palate. Diagnosis was made on the basis of immunohistochemical examination of biopsies. Both patients underwent CHOEP chemotherapy followed by radiotherapy. Nasal NK/T-cell lymphoma is an aggressive type of non-Hodgkin's lymphoma with specific clinicopathologic features. Combining chemotherapy with radiotherapy in patients with advanced stage of the disease does not seem to improve survival compared with radiotherapy alone, which is the treatment of choice especially for localized stages. Nasal NK/T-cell lymphoma is rare. Diagnosis is based on immunohistochemical examination. Treatment includes chemotherapy and radiotherapy. This lymphoma has a poor overall prognosis, even with appropriate therapy.

[Ankylosing spondylitis associated with Still's disease: should it be considered a pathophysiologic link or a simple association?] / Spondylarthrite ankylosante et maladie de Still: un lien physiopathologique ou une simple association?

Ankylosing spondylitis is a chronic inflammatory rheumatism; it is part of the group of spondyloarthrites. General signs such as fever and weight loss are of little importance. Adult Still's disease is a rare systemic condition, a diagnosis of exclusion commonly characterized by high hectic fever, rash, arthritis and various systemic manifestations. Few cases of ankylosing spondylitis associated with adult Still's disease have been described in the literature. We here report the case of a 31-year old patient followed up for ankylosing spondylitis presenting with fever which had lasted for a long time and clinico-biological signs compatible with adult Still's disease. A possible pathophysiologic link between the two diseases may be suggested, even if their simultaneous occurrence has been rarely reported in the literature.

[Diagnosis of lymphocytopenia]. / Orientation diagnostique devant une lymphopénie.

Lymphopenia is defined as a peripheral lymphocyte count lower than 1500/mm3 in adults and 4500/mm3 in children younger than eight months of age. We propose a classification of lymphopenia according to the mechanism involved: lymphocyte production defects, including primary immune deficiencies and immune deficiencies secondary to malnutrition or zinc deprivation; excess catabolism, due to causes including radiotherapy, chemotherapy, immunosuppressive therapy, HIV infection, and systemic lupus erythematosus; abnormal lymphocyte trapping, including mainly splenomegaly, certain viral infections, septic shock, extended burns, systemic granulomatosis, and corticosteroids; other causes of lymphocytopenia, with mechanisms that remain poorly understood: ethnicity (Ethiopians), lymphoma, renal insufficiency, and idiopathic CD4 lymphocytopenia.

Challenges for lupus management in emerging countries.

In emerging countries, systemic lupus erythematosus (SLE) has been associated with several unfavorable outcomes including disease activity, damage accrual, work disability and mortality. Poor socioeconomic status (SES) and lack of access to healthcare, especially in medically underserved communities, may be responsible for many of the observed disparities. Diagnostic delay of SLE or for severe organ damages (renal involvement) have a negative impact on those adverse outcomes in lupus patients who either belong to minority groups or live in emerging countries. Longitudinal and observational prospective studies and registries may help to identify the factors that influence poor SLE outcomes in emerging countries. Infection is an important cause of mortality and morbidity in SLE, particularly in low SES patients and tuberculosis appears to be frequent in SLE patients living in endemic areas (mainly emerging countries). Thus, tuberculosis screening should be systematically performed and prophylaxis discussed for patients from these areas. SLE treatment in the developing world is restricted by the availability and cost of some immunosuppressive drugs. Moreover, poor adherence has been associated to bad outcomes in lupus patients with a higher risk of flares, morbidity, hospitalization, and poor renal prognosis. Low education and the lack of money are identified as the main barrier to improve lupus prognosis. Newer therapeutic agents and new protocols had contributed to improve survival in SLE. The use of corticoid-sparing agents (hydroxychloroquine, methotrexate, azathioprine and mycophenolate mofetif) is one of the most useful strategy; availability of inexpensive generics may help to optimize access to these medications.

Thromboprophylaxis use and concordance with guidelines among medical and surgical patients in Morocco.

INTRODUCTION: No data are available on thromboprophylaxis use in Morocco. Our aim was to characterize patients at risk of venous thromboembolism and assess the rate of appropriate thromboprophylaxis. MATERIALS AND METHODS: This was a national, observational, multicentre survey of venous thromboembolism risk and thromboprophylaxis use in hospitalized patients. Data were collected on a predefined date in three university hospitals in Morocco using a standardized pre-printed form. Thromboembolic risk was assessed according to the American College of Chest Physicians (ACCP) 2008 guidelines. Patients were classified as "thromboprophylaxis indicated" or "thromboprophylaxis not indicated". RESULTS: 784 patients were analysed: 307 (39.2%) medical and 477 (60.8%) surgical. 421 (53.7%) were female. Medical patients were older than surgical patients (57.6 ± 11.5 vs. 46.2 ± 16.9 years, p<0.0001) and were more likely to have risk factors for thromboembolism (50.5% vs. 45.7% of patients, p=NS). 57% of patients without contraindications or bleeding risk were at risk of thromboembolism according to ACCP guidelines and thromboprophylaxis was prescribed to 42.8% of these patients. In contrast, 7.4% of patients with no thromboembolic risk also received thromboprophylaxis (proportion agreement: 61.0%; Kappa=0.296). Over half (54.5%) of medical patients at risk of thromboembolism did not receive thromboprophylaxis whereas 6.3% of those with no risk did receive it (proportion agreement: 76.4%; Kappa=0.433). These figures were 57.9% and 9.2%, respectively, for surgical patients (proportion agreement: 52.7%; Kappa=0.191). Thromboprophylaxis was given to 19.2% of patients with contraindications or a bleeding risk. CONCLUSIONS: Educational initiatives are imperative to inform doctors about appropriate thromboprophylaxis.

Bilateral coxitis in scleroderma-polymyositis overlap syndrome.

Joint manifestations in scleroderma (Scl) and polymyositis (PM) are dominated by inflammatory arthralgia. Arthritis is less common and preferentially affects the hands, wrists, knees, and ankles. Involvement of the hip has been rarely reported in the literature. We report a case of coxitis diagnosed in a patient suffering from scleroderma-polymyositis overlap syndrome successfully treated by ultrasound-guided infiltration of triamcinolone hexacetonide.

Magnetic resonance imaging findings within the posterior and lateral columns of the spinal cord extended from the medulla oblongata to the thoracic spine in a woman with subacute combined degeneration without hematologic disorders: a case report and review of the literature.

INTRODUCTION: Subacute combined degeneration of the spinal cord is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication of vitamin B12 deficiency. Subacute combined degeneration without anemia or macrocytosis is rare. CASE PRESENTATION: We present a case of cobalamin deficiency in a 29-year-old Moroccan woman who presented with subacute combined degeneration without evidence of anemia or macrocytosis. Magnetic resonance imaging of the spinal cord demonstrated abnormal hyperintense signal changes on T2-weighted imaging of the posterior and lateral columns from the medulla oblongata to the thoracic spine. A diagnosis of subacute combined degeneration of the spinal cord was considered and confirmed by low serum cobalamin. The patient was treated with vitamin B12 supplements and showed improvement in her clinical symptoms. CONCLUSION: Physicians should diagnose subacute combined degeneration in patients early by having a high index of suspicion and using diagnostic tools such as magnetic resonance imaging.

Visceral leishmaniasis in 26 HIV-negative adults.

BACKGROUND: Visceral leishmaniasis is a notifiable parasitic disease that had increased in incidence in our region on the past few years. It is common in children. In adults, it occurs more on a background of immunodeficiency, and frequently with incomplete clinical manifestations, making the diagnosis complicated. FINDINGS: The aim of our study is to reveal different features of visceral leishmaniasis in adults, through the analysis of its epidemiological, clinical and biological parameters, in a group of 26 patients. No one was infected with HIV or under immunosuppressive therapy Clinical presentation was generally conservative, but there was few differences in adults compared to children, concerning both the clinical symptoms and the laboratory parameters. Diagnosis was provided by direct examination of bone marrow smears in 24 cases (sensitivity 92%), and anti-leishmanial serology in the others. CONCLUSION: We should think to the diagnosis of VL even if the patient is not known immunocompromised, and even if the clinical is incomplete, to avoid a delay of care which can lead to serious complications.

Hemichorea-hemiballismus as an initial manifestation in a Moroccan patient with acquired immunodeficiency syndrome and toxoplasma infection: a case report and review of the literature.

Neurologic signs and symptoms may represent the initial presentation of AIDS in 10-30% of patients. Movement disorders may be the result of direct central nervous system infection by human immunodeficiency virus (HIV) or the result of opportunistic infections. We report the case of a 59 years old woman who had hemichorea-hemiballismus subsequently found to be secondary to a cerebral toxoplasmosis infection revealing HIV infection. Movement disorders, headache and nausea were resolved after two weeks of antitoxoplasmic treatment. Brain MRI control showed a marked resolution of cerebral lesion. Occurrence of hemichorea-ballismus in patient without familial history of movement disorders suggests a diagnosis of AIDS and in particular the diagnosis of secondary cerebral toxoplasmosis. Early recognition is important since it is a treatable entity.

Short- and long-term therapeutic effects of thermal mineral waters in knee osteoarthritis: a systematic review of randomized controlled trials.

The objective of the study was to evaluate short- and long-term therapeutic effectiveness of natural thermal mineral waters in patients with knee osteoarthritis (OA). We performed a systematic review of randomized controlled trials (RCTs) testing efficacy of thermal mineral water for treating patients with knee OA. Trials were identified by systematic searches of PubMed, Cochrane Central Register of Controlled trials, and Amed. We used the MeSH terms balneotherapy, balneology, and mineral water in combination with knee and osteoarthritis. Literature screening and data extraction were performed in duplicate. Nine RCTs satisfied the inclusion criteria, all published as full journal articles. Trial duration ranged from 10 to 24 weeks (median 15.33 +/- 5.56 weeks). The final sample included 493 patients who provided data at the ends of the studies. All interventions that were used in these trials found out an improvement in pain and functional capacity, which were sustained until week 24. No serious adverse events were reported to be associated with thermal mineral waters treatment. This work provide the most current and comprehensive review of the existing evidence of short- and long-term therapeutic effects of thermal mineral waters in knee OA. Additional RCTs with similar intervention comparisons and outcome measures, bigger sample size, and longer follow-up are required to confirm these results and to assess the biological effect of thermal mineral waters in patients with knee OA.