Mounier-Kuhn syndrome and tracheopathia osteoplastica in the same patient with respiratory insufficiency

SummaryMounier-Kuhn syndrome (MKS) or tracheobronchomegaly, and tracheopathia osteoplastica (TO) are rare diseases. MKS is a clinical entity characterized by abnormal dilatation of the trachea and main bronchi. TO is a clinical and pathologic condition characterized by multiple submucosal cartilaginous and osseous nodules lining the upper respiratory tract. Some people with MKS manifest recurrent pneumonia or chronic cough with sputum production, hemoptysis or dyspnea. Occasionally, it is found in asymptomatic individuals. CT scan imaging is the key tool for the diagnosis when showing a dilatation of the trachea and main bronchi. TO is usually benign and asymptomatic, frequently diagnosed incidentally during intubation. Flexible fiberoptic bronchoscopy and laryngoscopy are the key diagnostic tools which typically demonstrate irregular spicules of submucosal bone and cartilage projecting into the tracheobronchial lumen and causing various degrees of airway obstruction. When this aspect is found bronchial biopsy is not mandatory. We report the case of a 26-year-old man with features of both MKS and TO who presented a previous history of productive cough, recurrent pneumonia and dyspnea; admitted for pulmonary infection resulting in a respiratory insufficiency. MKS was diagnosed by CT scan and TO by fiberoptic bronchoscopy. To our knowledge, this is the first time these pathologies are reported in the same patient, and our patient is the second one having MKS who presented with respiratory insufficiency

En tuff väg tillbaka : En kvalitativ studie om före detta kriminellas perspektiv på inkludering i samhället.

Syftet med denna undersökning är att skildra före detta kriminellas perspektiv på inkluderingsprocessen till samhället efter att ha varit frihetsberövad. Denna kvalitativa undersökningen har en pedagogisk utgångspunkt där resultatet har analyserats utifrån en läroteori; det biografiska lärandet. Detta för att se hur förändringsprocessen av att byta livsstil, från kriminell till laglydig samhällsmedborgare, kan se ut. Deltagarna för denna undersökningen berättar hur denna process inte är enkel däremot finns det företeelser som har underlättat deras inkludering. Allt från myndigheters bemötande till att ta ansvar för sina tidigare handlingar men också att tillhöra ett sammanhang som exempelvis religiösa grupper, kamratföreningar eller en familj

Rotational and Gas Temperature Measurements for He-C Plasma: Application to Heterogeneous Carbon Nanotubes Synthesis

International audienc

Bleeding and Thrombosis in a Patient with Secondary Antiphospholipid Syndrome

Antiphospholipid antibodies have been associated with occurrence of arterial and venous thrombotic events and fetal loss, which together constitute the antiphospholipid syndrome (APS). However, bleeding is rare in this syndrome. We report a case of systemic lupus erythematosus (SLE) with APS complicated simultaneously by thrombotic and hemorrhagic events. A 34-year-old woman was a known case of diffuse proliferative lupus nephritis associated with APS, on treatment with corticosteroids, cyclophosphamide and anticoagulants. She presented in February 2004 with severe anemia, menorrhagia, gingival bleeding and acute loss of vision in the left eye. Investigations revealed a hematoma in the psoas muscle with thrombosis of the inferior vena cava and occlusion of the retinal vein. Blood tests revealed a strongly positive lupus anticoagulant, factor XI deficiency (35%) and decrease of free protein S (44%). Factor XI inhibitor, anti-prothrombin, and anti-protein S antibodies were absent. The patient was treated with corticosteroids and six pulses of cyclophosphamide, which resulted in a rapid disappearance of bleeding, reduction of hematoma and normalization of hematological abnormalities. She was maintained on corticosteroids, azathioprine and anticoagulant agents were introduced. After a follow-up of 28 months, there was no recurrence of bleeding, the thrombosis had resolved, and there was a decrease in the levels of circulating anticoagulant as well as anticardiolipin antibodies

Therapeutic efficacy of a biosimilar epoetin alfa in hemodialysis patients

Anemia is a frequent complication in patients with chronic kidney disease. However, human recombinant erythropoietin (rHu-EPO) has revolutionized the management of anemia in chronically dialyzed patients. Epomax ® is a new rHu-EPO alfa manufactured in Tunisia (Medis Laboratories). The aim of this study was to evaluate the efficacy and tolerance of Epomax ® in chronic hemodialysis (HD) patients in a phase-III, multicenter, clinical trial. Fiftythree HD patients (mean age 47.7 ± 13 years) who received a stable dose of rHu-EPO (Hemax ® , a rHu-EPO alfa manufactured by Biosidus Laboratories) subcutaneously were switched to Epomax ® via the same route of administration. At baseline, the mean systolic pressure was 132 ± 18 mm Hg and the mean diastolic pressure was 79 ± 8 mm Hg. The mean blood hemoglobin was 10.2 g/dL and the median ferritin level was 667 ng/mL. After a follow-up of 43 days, the mean blood hemoglobin was 10.5 g/dL under the effect of Epomax ® . There was no significant difference in the mean hemoglobin levels between the treatments with both drugs. Few adverse events were reported during the study. We conclude that Epomax ® was effective at maintaining the hemoglobin levels at target concentrations and was well tolerated in HD patients

The diagnosis of tuberculosis in dialysis patients

The incidence of tuberculosis (TB) is high in patients undergoing chronic dialysis than it is in the general population. The diagnosis of TB is often difficult and extrapulmonary involvement is predominant. This study investigates the spectrum of clinical presentations and outcome in dialysis patients during a nine-year period. TB was diagnosed in 41 patients. Anti-TB drugs, adverse effects of therapy, and outcome were noted. Thirty-eight patients (92.6%) were on hemodialysis and three were on peritoneal dialysis (7.3%). The mean age at diagnosis was 50.8 years and the male/female ratio was 1.16. Four patients had a history of pulmonary TB. Extrapulmonary involvement was observed in 32 (78 %) patients. The bacteriological confirmation was made in 41.46% and histological confirmation was made in 26.83%, and in the rest, the diagnosis was retained on the criterion presumption. Nineteen patients (46.34%) developed adverse effects of antitubercular drugs. Eight patients (19.51%) died during the study from TB or adverse effects of treatment. Low urea reduction ratio and female sex were associated with poor prognosis in our study. The clinical manifestations of TB in patients on dialysis are quite nonspecific, making timely diagnosis difficult, and delaying the initiation of curative treatment, which is a major determinant of the outcome

A case of renal granulomatosis with polyangiitis following intravesical bacillus Calmette-Guérin therapy

Various adverse reactions may occur after intravesical bacillus Calmette-Guérin (BCG) therapy. Although the virulence of attenuated BCG is low, serious complications such as bacterial cystitis, bladder contractures, granulomatous prostatitis, epididymitis, orchitis, and systemic reactions such as fever and malaise have been described. Disseminated granulomatosis such as hepatitis and pneumonitis have also been described, but are rare. We report here the case of a 67-year-old patient who presented with renal granulomatosis with polyangiitis following intravesical BCG therapy for superficial bladder tumor. The biological evaluation revealed the presence of perinuclear anti-neutrophil cytoplasmic antibodies with specificity for antimyeloperoxidase. Renal biopsy specimen revealed pauci-immune crescentic glomerulonephritis with segmental glomerular necrosis, presence of granulomas and no evidence of any caseating necrosis. He received antituberculosis drugs in addition to corticosteroids and cyclophosphamide without any improvement of the renal function

Osteomalacia complicating renal tubular acidosis in association with Sjogren′s syndrome

Renal involvement in Sjogren′s syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer′s test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L

Disseminated cryptococcosis as a complication of lupus nephritis

Cryptococcus neoformans is an opportunistic fungal infection affects predominately the central nervous system in HIV patients and patients with other immunocompromised states. It has rarely been described in immunocompetent patients. It is a serious infection with a high of mortality rate. We describe a case of a 48-year-old patient diagnosed with lupus nephritis treated with corticosteroids and mycophenolate mofetil who developed central nervous cryptococcosis complicated by septicemia. She died despite the use of antifungals. Cryptococcal infection is an uncommon, but often a fatal complication of systemic lupus erythematosus. Timely diagnosis and effective antifungal therapy could improve its prognosis