Unusual onset of a case of chronic recurrent multifocal osteomyelitis.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a rare condition that commonly affects the clavicle and pelvis. CASE PRESENTATION: We report here a case a 12 years old girl with CRMO arising with recurrent episodes of left supraorbital headache, followed by the appearance of a periorbital dyschromia. Magnetic resonance imaging (MRI) of the skull and orbits revealed an important subacute inflammatory process. Few months after, the child presented a painful swelling of the left clavicle; the histological examination of the related biopsy allowed to establish the diagnosis of CRMO. CONCLUSION: CRMO presenting as acute headache involving neurocranium is rare; to our knowledge this is the first recognized case in the world literature. This pathological condition is frequently misdiagnosed as infection or neoplasm and needs a deep investigation for the differential diagnosis. The physical, laboratoristic and instrumental diagnostic investigations of the patient and the treatment employed are described in detail.

Cholestasis as a presenting feature of acute Epstein-Barr virus infection.

Biochemical evidence of hepatic involvement in Epstein-Barr virus disease is common but clinical features of cholestasis are rare in children. We present three children with cholestasis as a presenting feature of Epstein-Barr virus disease.

Immunological evaluation of patients with beta-thalassemia major.

Abnormalities in the immune system and zinc homeostasis in patients with beta-thalassemia major (TM) have been reported. Since zinc ion is essential for the efficiency of the immune system and is required to induce biological activity to thymulin (Zn-FTS), a biochemically defined thymic hormone, we investigated the plasma levels of zinc and both active thymulin (Zn-FTS) and total zinc saturable thymulin (Zn-FTS+FTS) in 18 patients with TM aged between 2 and 31 years and 22 normal controls of the same age. Inhibitory molecules anti-thymulin and the distribution of lymphocyte subsets were also analyzed. Patients with TM presented significantly lowered plasma zinc and thymulin levels when compared to normal subjects. The significant enhancement of the active form of the hormone after zinc addition in vitro suggests that low thymulin values found in TM are due not to a thymic failure in synthesizing and secreting the thymic hormone, but a defect in zinc saturation of the hormone. An impairment of cell subset distribution was also demonstrated. This study shows that zinc and thymulin deficiency contribute to the complex mechanisms underlying immune dysfunction in TM.

Bartonella henselae and inflammatory bowel disease.

Sustained fever and increased thickness of the distal ileum on ultrasound suggested Crohn's disease in an adolescent boy. Bartonella henselae infection was diagnosed by specific serology and the patient recovered. Ileitis could be related to B. henselae infection.

Pseudoinfectious mononucleosis: a presentation of Bartonella henselae infection.

Six children presented during one year with clinical features of infectious mononucleosis, but with laboratory findings of leucocytosis with neutrophilia, increased erythrocyte sedimentation rate, and hypergammaglobulinaemia. Serology for Epstein-Barr virus, cytomegalovirus, adenovirus, and Toxoplasma gondii was negative, while anti-Bartonella henselae IgM with high IgG titre (>/=1/1024) was present in all. All children had contact with kittens. No specific treatment was administered and all recovered.

Widening of the clinical spectrum of Bartonella henselae infection as recognized through serodiagnostics.

UNLABELLED: The recently improved diagnostics have widened, in children, the spectrum of clinical manifestations recognisable as Bartonella henselae infection. We report here the clinical features of 20 (14 males) consecutive children with serologically proved B. henselae infection observed within 12 months in the Paediatric Department of the University of Pisa. The patients had a mean age of 7 years 4 months (range 1.1-14.1 years). All children but one had a history of contact with kittens. Clinical manifestations included regional lymphadenopathy in 14 patients, representing in five the only clinical manifestation at onset, infectious mononucleosis-like syndrome in six, erythema nodosum in three, and Parinaud oculoglandular syndrome in one. In five patients a severe disorder was first suspected: fever of unknown origin in two with multiple hepatosplenic granulomatosis in one; osteolytic lesion suggesting bone neoplasm, marked inguinal lymph-node enlargement, suggesting Burkitt lymphoma, and an acute encephalopathy in one each. Bartonella henselae IgG antibody was positive in all patients with a titre ranging from 1:128 to 1:8590. IgM antibody was present in all except one child with an IgG titre of 1:2048. All patients recovered, some spontaneously. CONCLUSION: Bartonella henselae infection is frequent in Tuscany and probably underdiagnosed due to the high frequency of atypical onset of the clinical manifestations. An accurate clinical history and a reasonably wide use of the serological test may allow a rapid and accurate diagnosis, reassuring the family of the patient and avoiding invasive and expensive diagnostic procedures.

[Outpatient neuropsychiatric consultation in a pediatric hospital. Clinical considerations]. / Un'esperienza di consulenza neuropsichiatrica ambulatoriale in un reparto di pediatria. Considerazioni cliniche.

BACKGROUND: In this study, personal experience as neuropsychiatric consultants in a pediatric hospital is reported. Infancy and adolescence neuropsychiatrics' and pediatricians' common aim is to evaluate a child in his entirety and to protect his physical and mental health. METHOD: 327 patients (147 males and 180 females) aged ranged 0 to 18 years, address to hospital by their pediatricians from January 1993 to January 1998 have been evaluated. RESULTS: Mental disorders have been diagnosed in 95% of the examined population (mental disorders classified by DSM IV criteria) the way in which mental disorders were distributed according to age and sex has been analyzed, and the therapeutic approaches discussed. Neuropsychiatric advice is requested especially for somatic and developmental disorders. Sex distribution is equal, apart from conduct disorders and pervasive developmental disorders, which are more frequent in males. Somatic, anxiety and mood disorders are more evident in children older than 6 years, developmental and neurological disorders are more evident in younger children. CONCLUSIONS: Outpatient advice is a valid help to screen neuropsychiatric disorders and it offers the possibility to carry out short and effective evaluations and controls with a significant reduction of costs for medical assistance.

Systemic Bartonella henselae infection with hepatosplenic involvement.

BACKGROUND: Systemic manifestations of Bartonella henselae infection are rare in the immunocompetent host. The infection generally has initial symptoms of prolonged fever and multiple granulomatous lesions in liver and spleen. METHODS: Retrospective analysis of the records of all patients with hypoechogenic lesions in the liver and/or spleen diagnosed from 1990 through 1996 in three pediatric clinics in northern Italy. RESULTS: Among the 13 patients reviewed, 9 had evidence of B. henselae infection and hepatosplenic involvement: five had prolonged and unexplained fever lasting from 3 to 16 weeks, and four had typical cat-scratch disease and peripheral lymphadenitis. All patients had increased sedimentation rate and normal aminotransferase serum activity. Five children had a liver biopsy, by laparotomy in three and by needle in two. In all, the predominant liver lesion was a necrotizing granuloma. All patients were treated with broad-spectrum antibiotics. Fever lasted from 3 to 16 weeks, and hepatic and splenic lesions resolved in all with residual splenic calcification in one. CONCLUSIONS: Systemic B. henselae infection represents an important cause of inflammatory hypoechogenic hepatosplenic lesions in children. Serology provides rapid diagnosis, avoiding multiple and invasive investigations. Hepatosplenic involvement can be found even in children with typical cat-scratch disease without apparent systemic manifestations. The frequency of liver and/or splenic involvement in cat-scratch disease is probably underestimated.