Liver Involvement in Patients With PiZZ-Emphysema, Candidates for Lung Transplantation.

Information about the prevalence and nature of liver disorders in adults with alpha1-antitrypsin deficiency is scarce. At our center, systematic liver biopsy screening is part of the evaluation before lung transplantation (LT) in the emphysema patients with the PiZZ phenotype. Our aim was to report our experience with this prospective screening. Clinical, liver function, and imaging parameters as well as liver histology data were analyzed for 23 consecutive adult patients with PiZZ severe emphysema referred to our center for consideration of LT from 2006 to 2014. Overall 20 (87%) featured chronic liver disease characterized by a chronic inflammation and/or a significant portal fibrosis on histology. Two of the 23 patients (8.7%) had septal fibrosis according to the Metavir and Ishak scores and met our definition of severe chronic liver disease. They were both clinically asymptomatic with normal liver function tests. On abdominal ultrasonography, the liver appeared normal in one patient and with abnormal contours in the other. Our data indicate that in adults with PiZZ-related emphysema being evaluated for LT, most patients had some histologic involvement. The prevalence of severe liver dysfunction is <10%.

Lytic bone lesions in lupus-associated myelofibrosis.

Myelofibrosis is associated with numerous causes other than hematologic malignant neoplasms, including autoimmune diseases. We describe a 44-year-old woman who suffered bone lytic lesions with extramedullary haematopoiesis in the setting of myelofibrosis associated with systemic lupus erythematosus.

[Epidemiology and delays in the management of 355 patients with lung cancer]. / Epidémiologie et délais de prise en charge de 355 patients atteints de cancer bronchique.

BACKGROUND: The epidemiology of patients with lung cancer in a Seine-Saint-Denis hospital are reported, as well as causes of diagnostic and therapeutic delays in their management. MATERIAL AND METHODS: Retrospective analysis of cases diagnosed from January 1, 1997 to December 31, 2003. RESULTS: Of 355 cases, 15.8% were women; the average age was 62 +/- 11. Mean smoking history was 50 +/- 24 pack years. Women were more likely to be non-smokers than men (16% and 1% respectively, p < 0.01) and were more likely to present at a young age (under the age of 50: 26.8% and 13.7% respectively, p < 0.05). Among women, adenocarcinoma was more frequent (41% vs. 25%, p < 0.05), and often presented with stage IV disease (74%). Squamous cell carcinoma occurred more frequently with increasing age (18.7% vs. 32.2% before and after the age of 60, p < 0.01). The median pre hospital, diagnostic and treatment delays were respectively 30, 10 and 9 days, the global delay from first symptom to treatment was 62 days. Surgery increased therapeutic delay by 20 days. CONCLUSION: Our results are complementary to those found in KBP-2000-CPHG study. Young women are diagnosed at a late stage. Influence of delays on prognosis is not proved and require others studies.

Complications following intravesical bacillus Calmette-Guerin treatment for bladder cancer: a case series of 22 patients.

BACKGROUND: Intravesical bacillus Calmette-Guerin (BCG) therapy is an effective and widely used treatment for superficial bladder carcinoma. Local complications are frequent whereas systemic complications are rare but can be serious, and their management is not well known. METHODS: We describe retrospectively the records of 22 patients treated in 3 infectious disease departments, for complications related to intravesical BCG therapy as treatment of bladder cancer. RESULTS: All the patients were male, with a median age of 68 years (range 56-88). Complications occurred after a median of 5 instillations (range 1-11) and were observed within 24 h following BCG instillation for 14 patients. Common symptoms were fever (n = 20), impaired general condition (n = 14), and shortness of breath (n = 7). Six patients had a systemic septic reaction leading to transfer into the intensive care unit for five of them. Lung infiltration was the most frequent presentation (n = 11). Mycobacterium bovis was isolated from only two patients, but histology showed the presence of a granuloma in nine patients. Antimycobacterial treatment was initialized in 17 patients; the outcome was favorable in 16 patients, with a median length of symptoms resolution of 22.5 days (range 5-425 days). Eleven patients received corticosteroids in addition to specific treatment and had a more rapid improvement. One patient died with disseminated BCGitis proved by biopsy. CONCLUSIONS: Complications following intravesical BCG therapy are rare but can be severe and fatal. Histology seems to be the method that contributes most in confirmation of the diagnosis. Antimycobacterial therapy is effective, and probably more efficient when combined with corticosteroids, but the regimen and duration of the treatment are not standardized.

[Pancreatic involvement, ascites and diarrhea in idiopathic hypereosinophilic syndrome]. / Atteinte pancréatique, ascite et diarrhée au cours d'un syndrome hyperéosinophilique idiopathique.

We report a new case of idiopathic hypereosinophilic syndrome with multivisceral digestive failure. After an erroneous diagnosis of pancreatic cancer, the pathological examination of pancreaticoduodenectomy specimen demonstrated pancreatic fibrosis with eosinophilic infiltration without gastritis or duodenitis. The diagnosis of idiopathic hypereosinophilic syndrome was made three months later upon the classical criteria: a) blood eosinophilia of 1.5 G/L or more, persisting for more than 6 months; b) lack of evidence for any other recognised cause of eosinophilia: c) multiple organ systemic involvement: rheumatologic, cutaneous and digestive (pancreatitis, ascites and diarrhoea): d) previous history of allergic disease and increased plasmatic IgE levels; e) absence of leukemic markers. This case emphasises the difficulty in classifying eosinophilic infiltration of the gut and the possibility of transitional forms between eosinophilic gastro-enteritis and idiopathic hypereosinophilic syndrome. We argue that in case of eosinophilic infiltration of the gut, systematic research of multiple organ systemic involvement is mandatory.

[Value of rectosigmoidoscopy with bacteriological culture of colonic biopsies in the diagnosis of post-antibiotic hemorrhagic colitis related to Klebsiella oxytoca]. / Intérêt de la rectosigmoïdoscopie avec culture bactériologique de biopsies coliques dans le diagnostic des colites hémorragiques post-antibiotiques associées à Klebsiella oxytoca.

UNLABELLED: We report 7 cases of antibiotic-associated and hemorrhagic colitis due to Klebsiella oxytoca. The diagnosis was performed by sigmoidoscopy with bacteriological biopsy culture. PATIENTS AND METHODS: Nine patients, aged 25-83 years, were hospitalized from February 1993 to October 1995 with hemorrhagic acute diarrhea following antibiotic treatment: amoxicillin (n = 3). amoxicillin-clavulanic acid (n = 4), cephalosporine (n = 2). All patients have had two stool samples cultures including Clostridium difficile toxin detection. Sigmoidoscopy with collection of biopsy specimens for bacteriological cultures was performed routinely. RESULTS: Endoscopic findings established the diagnosis of colitis in all cases: rectitis (n = 1), diffuse left colitis (n = 4), segmental left colitis (n = 4). The lesions were erythematous and purpuric (n = 5) or ulcerative (n = 4). Stool culture was normal in all cases but Klebsiella oxytoca was isolated in 7 cases (78%). CONCLUSIONS: Sigmoidoscopy and bioptic microbiology ensured the diagnosis of antibiotic-associated hemorrhagic colitis due to Klebsiella oxytoca.

[CD8 hyperlymphocytosis syndrome and human immunodeficiency virus infection: 5 cases]. / Syndrome d'hyperlymphocytose CD8 et infection par le virus de l'immunodéficience humaine: cinq observations.

A retrospective study on 202 consecutive patients with HIV infection was reviewed. A particular syndrome with blood CD8 lymphocytosis > 1 500/mm3, associated with a diffuse lymphocytic infiltrate histologically proved in the tissue of different organs was present in five patients. Clinical findings were variable, depending on the location of visceral infiltrate by activated, polyclonal lymphocytes of CD8 phenotype: interstitial pneumonia (n = 3), parotid gland enlargement with sicca syndrome (n = 2), pseudo-tumoral splenomegaly (n = 1), peripheral neuropathy (n = 1), superficial generalized lymphadenopathy (n = 5). This syndrome occurred early during HIV infection. All patients had a blood CD4 lymphocyte count > 200/mm3. This disorder is a host immune response, sometimes associated with the presence of some HLA antigens: HLA-DR5 or HLA A1 B8 DR3. Whether this immune response is specific or not, whether outcome of HIV infection depends on hyper CD8 lymphocytosis remains to be proved.

[Histopathology of cutaneous lesions]. / Histopathologie des lésions cutanées.

Kaposi's sarcoma in AIDS syndrome differs in many ways from the typical nodular lesions observed in older patients. Clinical lesions are usually multiple with no predilection for the lower extremities; they usually present as small macules or patches with little or no infiltration, pink rather than deeply violaceous; indeed their clinical recognition may be difficult. Histological features of early patches of Kaposi are subtle: they are not as obvious as in well-developed nodular lesions: however certain precise clues exist which permit a correct diagnosis to be made.

[Clear cell sarcoma of the kidney in a young adult]. / Sarcome à cellules claires du rein chez un jeune adulte.

We report a case of clear cell sarcoma of kidney (CCSK), in a young adult. This highly malignant renal tumor usually arises in children of one or two year old and represents 4% of renal tumors. The aggressivity of this tumor and its affinity to give bone metastasis imply to recognize it in order to institute appropriate chemotherapy.

[Solitary plasmacytoma of the trachea. Apropos of a case]. / Plasmocytome solitaire de la trachée. A propos d'un cas.

A case of solitary plasmacytoma of the trachea is reported. Immunohistochemical study is mandatory to prove a monoclonal immunophenotyping. Research for signs of systemic disease and follow up over a long period of time are indicated. Surgery alone, sometimes followed by irradiation, seem to be the treatment of choice. In most cases, complete resolution is observed.

[Multinucleated cell angiohistiocytoma. Report of 6 cases]. / Angiohistiocytome à cellules multinucléées. A propos de six cas.

Multinucleate cell angiohistiocytoma is a recently described entity. It is a benign vascular proliferation. Clinically, it is characterized by violaceous red papules, often mimicking Kaposi's sarcoma. Acral sites and face were the commonest sites. The six patient's age was between 41 and 64 years and sex ratio was equal. Microscopic features were an increased number of blood vessels together with mononucleated and multinucleated histiocyte-like cells with scalloped borders. Staining of mononucleated cells with CD68, anti vimentin and anti factor XIIIa antibodies emphasized a fibrohistiocytic origin. Loss of factor XIIIa expression in multinucleate cells gets clue to think that these cells are dedifferenciated.

[MALT lymphoma of the lacrimal apparatus (mucosa-associated lymphoid tissue)]. / Lymphomes lacrymaux de type MALT (Tissue Lymphoïde Annexé aux Muqueuses).

Mucosa-associated lymphoid tissue lymphoma can occur in various localizations. We describe the case of a patient presenting bilateral MALT lymphoma involving a lacrimal gland and a lacrimal duct. Biopsy specimens are required for histologic diagnosis. Such low-grade lymphomas are generally treated medically with radiotherapy or chemotherapy. Prognosis is generally favorable. A complete work-up is required to determine extension. These patients should be followed regularly.

[Oncocytoma of the caruncle. Anatomo-clinical study apropos of a case]. / Oncocytome de la caroncule. Etude anatomo-clinique à propos d'un cas.

A clinico-pathologic case of an oncocytoma of the caruncle is reported in a 60-year old male patient. Clinically a granular reddish painless tumor was observed in the caruncle. A total surgical excision was performed. The histopathological study established the diagnosis of oncocytoma of the caruncle. Although rare, this tumor should be recognized by ophthalmologists, specially in elderly, because of exceptional adenocarcinoma. The oncocytoma is cured by surgical excision.

[Fronto-naso-ethmoido-sphenoido-maxillo-orbital mucocele with ophthalmologic presentation]. / Mucocèle fronto-naso-ethmoïdo-sphénoïdo-maxillo-orbitaire à révélation ophtalmologique.

PURPOSE: We present a case of a large mucocele pressing the orbit forward and compared our findings with those reported by others. CASE REPORT: A 23-year-old woman was examined for an inflammatory tumor of the internal canthus. She had a 6/10 vision loss of the left eye, diplopia, and non-axial exophthalmos. CT scan and magnetic resonance imaging evidenced a huge sinus mucocele behind the orbit. Surgery using the paralateronasal approach was performed. Pathology confirmed the diagnosis of mucocele. DISCUSSION: Ophthalmic complications of mucoceles result from tumor growth leading to compressive optic neuropathy or even compression of the chiasma. A sinus mucocele should be suspected upon indirect clinical signs and lead to neuroradiological explorations. Magnetic resonance imaging reveals iso- or high signals on T1-weighted sequences and high signal on T2-weighted sequences. MRI evidences intracranial or orbital extension. CT scan reveal the degree of bone erosion. Prognosis is favorable after surgical treatment. It is important to correctly diagnose mucocele on the basis of clinical and neuroradiological findings in order to propose early surgery and prevent permanent visual loss by compressive optic neuropathy.

[Human Pasteurella multocida infections. Value of serological diagnosis]. / Les infections humaines à Pasteurella multocida. Intérêt du diagnostic sérologique.

In five human cases of Pasteurella multocida infection, high titres of specific antibodies were found by indirect haemagglutination (capsular antigens) and agglutination (somatic antigens). The specificity of the antibodies fitted with the serotypes of the isolated strains (three A 3 and two A 7). Subsequent changes in antibody titres in human beings were similar to those observed in animals. The risk of this animal-acquired infection being probably underestimated in medical practice, the usefulness of these tests in human beings is discussed.

[Intraductal mucinous tumor of the pancreas: report of a clinical case]. / Tumore intraduttale pancreatico mucinoso: a proposito di un caso clinico.

A 49-year-old diabetic patient with abdominal pain was found at ultrasonography and computed tomography to have a cystic mass in the head of the pancreas with dilatation of the main pancreatic duct. The head of the pancreas and the duodenum were removed surgically. Examination of the operative specimen showed chronic pancreatitis, dilatation of the main pancreatic duct, and impacted mucus in the secondary ducts with villous proliferation of the ductal epithelium, thus allowing a diagnosis of intraductal adenomatosis. There was no evidence of malignancy. The resection margin was involved, and consequently the remainder of the pancreas was removed six months after the initial surgical procedure. A review of the literature showed that intraductal adenomatosis tends to spread and carries a high risk of malignant transformation. Surgery is required because of the risk of pancreatic duct obstruction and pancreatic cancer. Intraductal papillary tumour of the pancreas shares many characteristic with other adenomatous proliferation of the gastrointestinal tract (colorectal villous adenoma, bile duct adenomatosis) including the presence of villous structures with increased mucus production, a tendency to spread massively, and a high risk of malignant transformation.