[Development and long-term prognosis of unexplained persistent inflammatory biologic syndromes]. / Evolution et pronostic à long terme des syndromes inflammatoires biologiques persistants inexpliqués.

PURPOSE: Unexplained inflammatory syndrome is a frequent and worrying condition in Internal Medicine. However, the long-term clinical outcome of these patients cannot be inferred from the literature. The aim of this study is to describe the long-term follow-up and the prognosis of a group of patients hospitalised for an inflammatory syndrome and discharged without causal diagnosis. METHODS: This retrospective study was carried out on 46 patients, 15 men and 31 women, aged 21 to 90 years, hospitalised between 1992 and 1999. Data concerning the hospital stay were obtained from the patients' medical record. Follow-up was performed by consulting the treating physician. RESULTS: The prognosis of these patients is fairly good. In one third of the cases, the inflammatory syndrome resolved spontaneously (n = 13). In the second third, a definite diagnosis was established after discharge (n = 14) and consisted mainly of chronic inflammatory diseases (n = 9), cured with a specific treatment. In the remaining third (n = 12), the inflammatory syndrome persisted, in clinically asymptomatic patients. CONCLUSION: These results suggest that the persistence of an inflammatory syndrome is not a poor prognostic factor. Thus we propose for patients discharged with an undiagnosed persistent inflammatory syndrome despite thorough investigations, a simple clinical and biological follow-up instead of repeated etiological investigations.

[Hungry bone syndrome after surgical treatment of severe primary hyperparathyroidism: about 3 cases]. / Hungry bone syndrome après traitement de l'hyperparathyroïdie primaire sévère.

Persistant hypocalcemia occurring after surgical treatment of severe primary hyperparathyroidism may be due to transient or permanent hypoparathyroidism but also to a bone disease. We report three cases of hypocalcemia after surgery of large parathyroid adenoma or hyperplasia in women. Plasma calcium, phosphate and PTH levels are in accordance with Hungry Bone Syndrome (HBS). HBS is related to both excessive bone demineralization and turn over. It is a major importance to distinguish HBS from surgical hypoparathyroidism in order to start early the appropriate treatment given for a long period.

[Adult Still's disease: an unrecognized cause of acute febrile hepatic cytolysis. Study of twelve patients]. / Maladie de Still de l'adulte: une cause méconnue de cytolyse hépatique aiguë fébrile.

OBJECTIVE: Certain liver test abnormalities have been described in adult Still's disease. The objective of the present study was to analyze their type and frequency. PATIENTS: In a 10 year retrospective study, patients were included if they fulfilled Kahn's and/or Yamaguchi's diagnostic criteria (median follow-up: 6.5 years). RESULTS: Twelve patients were selected. The median age was 25 years old and the sex ratio H/F was 2.7. Fever was present in 100% of patients and hepatomegaly in 41%. Liver test abnormalities were identified in 92% of patients: moderate cytolysis (level of transaminases between 2 and 5 N) (83%), severe cytolysis (level of transaminases > 5 N) (17%), cholestasis (elevated levels of GGT and/or alkaline phosphatase) (75%), and an increase in the LDH level (41%). All these liver abnormalities resolved spontaneously or during treatment (83%), within a median of 18 days. CONCLUSION: Our study confirms the high frequency of liver test abnormalities (> 2/3 of the patients) in adult Still's disease. These abnormalities are generally moderate and asymptomatic (3/4 of the cases), but severe cytolysis may exist. This emphasizes the need to consider a diagnosis of adult Still's disease in the presence of fever and elevated transaminase activity.

[Anemia caused by vitamin B 12 deficiency in subjects aged over 75 years: new hypotheses. A study of 20 cases]. / Anémies par carence en vitamine B12 chez le sujet âgé de plus de 75 ans: nouveaux concepts. A propos de 20 observations.

PURPOSE: New hypotheses have recently been developed on vitamin B12 deficiency and the frequently observed occurrence in the elderly subject of food cobalamin malabsorption, i.e., the non-dissociation of B12 and its carrier protein (ND B12), and the possibility of rectifying this imbalance by oral crystalline B12 supplementation. The aim of this study was therefore to confirm these hypotheses in a series of patients aged over 75 years with anemia due to B12 deficiency. METHODS: A retrospective study was carried out over a 5-year period on patients aged over 75 years presenting with megaloblastic anemia (hemoglobin [Hb] < 12 g/dL) and vitamin B12/cobalamin deficiency (B12 < 160 pg/mL). RESULTS: Twenty cases were analyzed. The average age of the patient population was 82.5 +/- 6 years, and the F/M sex ratio was 1:2. Mean Hb levels were 7.9 +/- 2.4 g/dL, mean serum B12 levels were 83 +/- 24 pg/mL, and mean homocysteinemic levels were 35 +/- 27 mumol/L. The diagnosis was as follows: food cobalamin malabsorption/ND B12 (n = 10), Biermer's disease/pernicious anemia (n = 5), malabsorption due to pancreatic insufficiency (n = 1), and low dietary B12 levels (n = 1). Disorders associated with ND B12 were: atrophic gastritis and Helicobacter pylori infection (n = 6), antacid or biguanide intake (n = 3), alcohol abuse (n = 2), or idiopathic syndrome (n = 2). In the patients who were followed up (n = 10), i.m. (n = 5) or oral (n = 5) administration of crystalline B12 resulted in the correction of hematological abnormalities. CONCLUSION: In the elderly subject, food cobalamin/ND B12 malabsorption appears to be the main cause of B12 deficiency, and is frequently associated with atrophic gastritis. In these cases, administration of oral crystalline B12 may be an efficient means of treating this disorder.

[Capillary hyperpermeability syndrome of the face]. / Syndrome d'hyperperméabilité capillaire de la face.

BACKGROUND: Capillary leak syndrome is a rare disorder, with various clinical features, characterized by episodes of diffuse edema. CASE REPORT: We report the case of a 83 year-old woman with capillary leak syndrome of the face characterized by recurrent episodes of edamata. We found a follicular non-Hodgkin's lymphoma and high levels of plasma interleukin (IL) 2 and IL 6. Regression of edema and remission of hematology disease have been obtained with corticoids and chloraminophene. DISCUSSION: This case illustrates that capillary leak syndrome may present as an edema of the face and may be in some cases considered as a paraneoplastic syndrome. The role of cytokines seems important. This case suggests that corticoids may be useful in the management of neoplasm induced capillary leak syndrome.

[Sepsis syndrome and bone marrow granulomatosis after intravesical instillation of BCG]. / Syndrome septicémique et granulomatose médullaire dans les suites d'une instillation intra vésicale de BCG.

BACKGROUND: BCG therapy is an effective treatment for superficial bladder carcinoma. Exceptionally, systemic disorders including hypersensitivity reactions or infections may occur. CASE REPORT: A 70-year-old man developed septicemia with bone marrow granulomatosis with a favorable course after intravesicle administration of bacillus Calmette-Guérin for bladder carcinoma. DISCUSSION: High grade fever, septicemia, hepatitis or pulmonary granulomatosis as well as bone marrow involvement are reported in less than 1% of all cases of BCG therapy. The pathogenic mechanisms are complex. Anti-tuberculous drugs are generally given.

[Rheumatoid purpura and intravenous immunoglobulins]. / Purpura rhumatoïde et immunoglobulines intraveineuses.

Henoch-Schönlein purpura is a vasculitis usually with a benign course. Abdominal symptoms occur in 70% of cases, with possible intussusception or intestinal perforation. There is no clear evidence of the efficacy of a treatment in complicated cases of Henoch-Schönlein purpura. Corticosteroids improve abdominal pain but they do not have any effect on renal involvement or prevention of relapses. Intravenous immunoglobulins have been efficient in some cases with recurrent abdominal symptoms or progressive renal lesions. We report the case of a 19-year-old patient with severe abdominal involvement and early renal manifestations of Henoch-Schönlein disease, rapid and sustained improvement was obtained by intravenous immunoglobulins given during 48 hours.

[Fatal hemorrhagic necrosis of pituitary macro-adenoma after a stimulation test]. / Nécrose hémorragique mortelle d'un macro-adénome hypophysaire après test de stimulation.

Pituitary stimulation tests are widely used to explore hypophyseal adenomas. There are few disadvantages, although a few cases of pituitary necrosis have been published. We report a new case with a dramatic outcome. A 30-year-old man with clinical signs of acromegalia and major visual disorders was found to have a voluminous macro-adenoma of the pituitary gland. Thirty minutes after beginning the stimulation test, the patient complained of major headache and experienced persistant vomiting for several hours. Brain magnetic resonance imaging the next day did not reveal evidence of pituitary necrosis. Sudden onset coma occurred one hour later. The CT scan demonstrated hemorrhagic necrosis of the adenoma. The patient died despite emergency surgery. Due to the risk of hemorrhagic necrosis of a pituitary adenoma, baseline assays may be sufficient for diagnosis in patients with clinical signs highly suggestive of pituitary oversecretion, especially when a voluminous tumor is involved. Rigorous clinical surveillance is required after stimulation tests.