Decreased serum levels of nutritional biochemical indices in healthy children with marginally delayed physical growth

Biochemical markers of nutritional status (albumin, transthyretin, insulin-like growth factor-I and zinc) were measured in slowly growing two- to five-year-old, low-income Parisian children whose weight-for-height or height-for-age z scores (WHZ or HAZ) were between — 1 and — 2 SD of the NCHS median. The results were compared to controls who were matched for age, sex, and ethnic origin with WHZ and HAZ between — 1 and + 2 SD. Mean serum levels of transthyretin, albumin and insulin-like growth factor-I and mean plasma zinc concentrations were significantly lower in the growth-impaired children than in the controls ( p = 0.002, p = 0.006, p = 0.015, and p = 0.035, respectively). While the height-retarded children had low mean serum insulin-like growth factor-I values, the weight-retarded subjects had decreased levels of albumin, transthyretin and zinc when compared to controls. Lower mean levels of nutritional markers in healthy, slowly growing children suggest that inadequate dietary intakes of zinc, protein and/or energy may result in marginal delays in weight and height gains.     

Staphylococcal scalded skin syndrome: An uncommon symptomatology revealing an immune deficiency

Primary immune deficiencies associated with hyper-IgE syndrome are rare diseases with clinical features dominated by recurring cutaneous and visceral bacterial infections, particularly infections due to Staphylococcus species. Most of these infections are associated with milder inflammation compared to normal. We report a primary immune deficiency associated with a hyper-IgE syndrome revealed by a staphylococcal scalded skin syndrome in a 5-year-old girl. The patient presented with a severe staphylococcal infection with extensive skin lesions and disseminated intravascular coagulation. She received intravenous fluids to compensate for fluid losses and anti-staphylococcal antibiotics. Coagulopathy was also corrected. However, the progression was rapidly fatal.     

Immunohistochemical analysis of c-erbB-2, Bcl-2, p53, p21WAF1/Cip1, p63 and Ki-67 expression in hydatidiform moles

Hydatidiform moles (HM) are characterized by an abnormal proliferating trophoblast with a potential for a malignant transformation. Similar to other human tumors, trophoblastic pathogenesis is likely a multistep process involving several molecular and genetic alterations. The study was performed to investigate the expression patterns of c-erbB-2 and Bcl-2 oncoproteins, p53, p21^WAF1/CIP1 and p63 tumor suppressor proteins and Ki-67 cell proliferation marker in HM.We conducted a retrospective study of 220 gestational products, including 39 hydropic abortions (HA), 41 partial HM (PHM) and 140 complete HM (CHM). The expression of c-erbB-2, Bcl-2, p53, p21^WAF1/CIP1, p63 and Ki-67 was investigated by immunohistochemistry on archival tissues. c-erbB-2 expression was observed in three PHM and 10 CHM. Bcl-2 immunostaining was significantly higher in PHM (61%) and CHM (70.7%) compared with HA (7.7%, p?=? 0.001 and p?<? 0.0001, respectively). p53 expression was stronger in CHM (73.6%) compared with PHM (24.4%, p < 0.0001) and HA (12.8%, p < 0.0001). p21^WAF1/CIP1 staining was observed as well in molar and non-molar gestations (p?>? 0.05). p63 immunoexpression was significantly described in CHM (85.7%) and PHM (78%) compared with HA (10.2%, p < 0.0001 and p = 0.0001, respectively). Ki-67 was significantly expressed in CHM (72.1%) compared with HA (46.2%, p = 0.005).Altered expression of Bcl-2, p53, p63 and Ki-67 reflects the HM pathological development. Immunohistochemical analysis is beneficial to recognize the HM molecular and pathogenic mechanisms. Furthermore, it could serve as a useful adjunct to conventional methods for refining HM diagnosis.     

Spectrum of autoantibodies other than anti‐desmoglein in pemphigus patients

Background Pemphigus is a life‐threatening autoimmune blistering disease mediated by autoantibodies against adhesion molecule of the skin. Its concurrence with systemic and organ‐specific autoimmune disease was described in case reports. Objectives To evaluate the presence of a broad spectrum of organ‐specific and non‐organ‐specific autoantibodies other than anti‐desmoglein antibodies in pemphigus patients. Patients and methods Serum samples were obtained from 105 pemphigus foliaceus (PF) patients, 51 pemphigus vulgaris (PV) patients and 50 controls. Both indirect immunofluorescence assay and ELISA were used to assess the presence of autoantibodies related to connective tissue diseases, autoimmune hepatitis, vasculitis, rheumatoid arthritis, coeliac disease, diabetes and thyroiditis. Results Significant difference was observed between the three groups for anti‐thyroglobulin antibodies in the pemphigus foliaceus group (18% vs. 4%, P = 0.03). A significantly higher occurrence of IgM anti‐cardiolipin (P = 0.03), IgG anti‐reticulin (P = 0.01) and IgG anti‐gliadin antibodies (P = 0.008) were observed in the PV group. Cases with more than four autoantibodies were frequently positives for both anti‐desmoglein 1 and anti‐desmoglein 3. Conclusion Autoantibodies other than anti‐desmoglein antibodies are not rare in pemphigus patients. Clinical and serological follow‐up of pemphigus patients with positive autoantibodies are needed to clarify their impact in disease evolution.     

Gougerot-Sjögren syndrome disclosed by MALT lymphoma of the salivary glands. Report of 3 cases

Sj?gren's syndrome is characterized by an increased risk of developing non-Hodgkin's lymphoma. The lymphoma is most frequently extra-nodal, preferentially affecting the salivary gland: low-grade MALT lymphoma. Conversely, underlying Sj?gren's syndrome has been recently identified by some authors in patients with non-Hodgkin's lymphoma. In the present report, we present three cases of Sj?gren's syndrome disclosed by low-grade salivary gland MALT lymphoma. The patients were all women aged 33, 38 and 52 years. Extension work-up revealed nodal and bone marrow involvement in one case and no evidence of disseminated disease in the two others. Using the European criteria, all of our patients had certain Sj?gren's syndrome. Labial salivary gland biopsy and immunopathological studies in newly diagnosed low-grade MALT lymphoma would be helpful in identifying the real frequency of this association.     

Childhood discoid lupus erythematosus: a Tunisian retrospective study of 16 cases

Discoid lupus erythematosus (DLE) is uncommon in children. The clinical features of childhood DLE are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high level of transition to systemic disease. We undertook a retrospective study of 16 children with DLE ranging in age from 2 to 15 years, seen over a 9-year period. Six were less than 10 years old at the onset of the disease. The sex ratio was equal. The frequency of childhood DLE was about 7% of the total number of DLE patients seen in our department. Photosensitivity was defined as a clinical history of induction or exacerbation of discoid lesions following sun exposure, and was present in 81% of patients. There was no progression to systemic lupus erythematosus (SLE); an average follow-up time was 10.5 months (2-30 months). We would like to emphasize the increased frequency of childhood DLE in our country and the importance of photosensitivity. However, follow-up data regarding transition to SLE is lacking, therefore we are unable to offer a prognosis to our patients.     

Tuberculous mesenteric lymphadenitis: a case report

The authors report a case of mesenteric tuberculous lymphadenitis which mimic a pancreatic cystadenoma. They emphasize ultrasound and CT scan features which lead to the recognition of tuberculosis and permit a percutaneous fine needle biopsy. Laparotomy seems the most reliable method for a positive diagnosis. The place of different imaging methods is discussed.     

MRI findings in the diagnosis of pseudotumoral humeral hydatid

Hydatidosis of bone is rare. Vertebral localizations predominate. Standard imaging may be misleading, suggestive of a malignant tumor or infection. We present the case of a patient with a humeral hydatid. This localization is very rare and presents an unusual radiographic aspect. Computed tomography produces an image suggestive of a tumor. The correct diagnosis can be established with MRI, particularly with the STIR sequence. In our patient, MRI enabled us to establish the diagnosis preoperatively and evaluate extension. MRI has been found to be highly contributive to the diagnosis of hydatidosis of bone and for assessment of intra- and peri-osseous extension.