Periostitis reactiva florida: caso clínico y actualización del tema / Florid reactive periostitis: case report and update

La periostitis reactiva florida es un cuadro clínico benigno pero con una apariencia clínica y radiológica, que unida a su baja frecuencia, puede inducir a confusión con cuadros más graves como infecciones o tumores óseos malignos. Su tratamiento consiste en la escisión local del tumor pero puede ser más agresivo desde el punto de vista del déficit funcional que genera la tumoración, como en el caso que exponemos. Presentamos la historia clínica, estudio de imagen ,histología y tratamiento de un paciente que presentó un cuadro de periostitis reactiva florida en la falange proximal del cuarto dedo de la mano derecha, en el que se realizó amputación del radio para mejorar la función de la mano (AU)

Florid reactive periostitis is a benign lesion which can be confused with other more serious lesions such as infections and malignant bone tumours because of its clinical appearance, radiological image and low frequency. Treatment consists of local excision of the tumour, but can be more aggressive in case of dysfunction. In this paper we present the medical history, radiographic image, histology and treatment of a patient who suffered florid reactive periostitis in the proximate phalange of fourth finger in the right hand. An amputation of forth ray was done in order to improve hand movement (AU)

Sarcoma osteogénico naso-maxilo-orbitario. Abordaje transcraneal / Naso-maxillo-orbital osteogenic sarcoma. Transcranial approach

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[Peutz-Jeghers syndrome]. / Síndrome de Peutz-Jeghers.

Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors. An aggressive approach of these patients seems to be necessary. We report the case of two brothers suffering from Peutz-Jeghers syndrome whose father and grandfather died as a consequence of the progression of an intestinal cancer related to the syndrome.

Infection with human papillomaviruses of sexual partners of women having cervical intraepithelial neoplasia.

Epidemiological studies show that human papillomaviruses (HPV) are strongly related to cervical cancer and cervical intraepithelial neoplasias (CIN). Unlike the case for women, there are no consistent data on the natural history of HPV in the male population even though these viruses are prevalent in males. We carried out a prospective study to assess the prevalence of HPV in males as well as the factors that determine such infections in 99 male sexual partners of women with CIN. The genitalia of the males were physically examined and subjected to peniscopy for the collection of scrapings which were subjected to the polymerase chain reaction and restriction fragment length polymorphism to detect HPV. Of the 99 males sampled, 54 (54.5%) were positive for HPV DNA, 24% of whom presented normal peniscopy, 28% presented evident clinical lesions and 48% isolated lesions consistent with subclinical infection. In the HPV-negative group, 53% showed normal peniscopy, 4% presented evident clinical lesions and 42% isolated lesions consistent with subclinical infection. The study detected a statistically significant association (P < 0.02, Pearson chi-square test) between HPV infection and both the mean number of sexual partners which a male had during his life and the mean number of sexual partners in the year prior to testing. Viral types 6 and 11 were most frequently encountered. The study shows that infection with HPV was frequent in male sexual partners of women with CIN.

Infection with human papillomaviruses of sexual partners of women having cervical intraepithelial neoplasia

Epidemiological studies show that human papillomaviruses (HPV) are strongly related to cervical cancer and cervical intraepithelial neoplasias (CIN). Unlike the case for women, there are no consistent data on the natural history of HPV in the male population even though these viruses are prevalent in males. We carried out a prospective study to assess the prevalence of HPV in males as well as the factors that determine such infections in 99 male sexual partners of women with CIN. The genitalia of the males were physically examined and subjected to peniscopy for the collection of scrapings which were subjected to the polymerase chain reaction and restriction fragment length polymorphism to detect HPV. Of the 99 males sampled, 54 (54.5 percent) were positive for HPV DNA, 24 percent of whom presented normal peniscopy, 28 percent presented evident clinical lesions and 48 percent isolated lesions consistent with subclinical infection. In the HPV-negative group, 53 percent showed normal peniscopy, 4 percent presented evident clinical lesions and 42 percent isolated lesions consistent with subclinical infection. The study detected a statistically significant association (P < 0.02, Pearson chi-square test) between HPV infection and both the mean number of sexual partners which a male had during his life and the mean number of sexual partners in the year prior to testing. Viral types 6 and 11 were most frequently encountered. The study shows that infection with HPV was frequent in male sexual partners of women with CIN.

Diaphyseal chondroblastoma in a long bone: first report.

Chondroblastoma is a rare benign bone tumor typically located in the epiphysis. We describe the first case of chondroblastoma arising in the diaphysis of a long bone. The patient was a 13-year-old girl who presented with pain over her right thigh. Radiographs showed a lytic lesion in the diaphysis of her right femur. A core biopsy and a subsequent surgical resection were performed.

Infección del tracto urinario por Shigella sonnei / Urinary tract infection caused by Shigella sonnei

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[Incidence of diabetes mellitus and prevalence of type 1A diabetes mellitus in children younger than 16 years old from the province of Ciudad Real]. / Incidencia en menores de 16 años y prevalencia de la diabetes mellitus tipo 1A en la provincia de Ciudad Real.

BACKGROUND: Because of the lack of data from our province, we performed the present study to determine the current situation and future evolution in our region of the most frequent chronic disease in childhood. OBJECTIVE: To evaluate the incidence of diabetes mellitus and the prevalence of type 1A diabetes mellitus in children younger than 16 years old from the province of Ciudad Real. MATERIAL AND METHODS: We performed an epidemiological, cross-sectional, observational study. The mark-release-recapture method was used to calculate exhaustivity. The patients were selected through surveys to primary care centers, hospital registries and diabetics associations in our province. All type 1A diabetics were included in the calculation of prevalence. Only diabetics with onset of symptoms in 1999 were included in the calculation of incidence. RESULTS: The incidence of diabetes mellitus in children younger than 16 years old was 26 per 100,000. Twenty-three children were diagnosed with the disease, with an exhaustivity rate of 88.5 %. The prevalence of type 1A diabetes mellitus in children younger than 16 years old was 2.1 per 1,000 and 0.42 per 1,000 inhabitants. The prevalence in the general population was 0.88 per 1,000 inhabitants. CONCLUSIONS: The incidence of diabetes mellitus in children younger than 16 years old and the prevalence of type 1A diabetes mellitus in the province of Ciudad Real are higher than expected and are the highest of currently known rates in Spain.

Incidencia en menores de 16 años y prevalencia de la diabetes mellitus tipo 1A en la provincia de Ciudad Real / Incidence of diabetes mellitus and prevalence of type 1a diabetes mellitus in children younger than 16 years old from the province of Ciudad Real (spain)

Antecedentes: Al carecer de estudios previos en nuestra provincia sobre diabetes mellitus tipo 1A se realizó el presente trabajo para conocer el estado actual en nuestro medio, y valorar en el futuro, la evolución de la enfermedad crónica más frecuente en la infancia. Objetivos: Determinar la prevalencia de la diabetes mellitus tipo 1 (DM1A) y la incidencia en menores de 16 años en la provincia de Ciudad Real. Material y métodos: Se trata de un estudio epidemiológico, observacional de tipo transversal. Se utilizó el método captura-marcaje-recaptura para el cálculo de la exhaustividad. Los pacientes se recogieron mediante encuesta en centros de salud, registros hospitalarios y asociaciones de diabéticos de la provincia. Para el cálculo de la prevalencia se incluyeron todos los diabéticos tipo 1A y para el cálculo de la incidencia se incluyen aquellos diabéticos en los que la enfermedad se inició en 1999. Resultados: La incidencia de diabetes mellitus en menores de 16 años ha sido de 26,0/100.000; se detectaron 23 niños, con una tasa de exhaustividad del 88,5%. La prevalencia de la DM1 en menores de 16 años es de 2,1/1.000 y de 0,42/1.000 habitantes de la provincia. En la población general la prevalencia es de 0,88/1.000 habitantes. Conclusiones: La incidencia de la diabetes mellitus en menores de 16 años y la prevalencia de la DM1A en la provincia de Ciudad Real es mayor de la esperada, siendo la mayor de las conocidas en España en la actualidad (AU)

Ewing's sarcoma of the mandible: radiologic features with emphasis on magnetic resonance appearance.

Ewing's sarcoma is an uncommon malignancy that usually occurs in children. A case of Ewing's sarcoma of the mandible is presented, and the radiologic appearance is described, with special consideration given to the magnetic resonance imaging features.

[Permanent neonatal diabetes associated with other anomalies]. / Diabetes neonatal permanente asociada ahipotiroidismo, sordera y rasgos dismórficos.

Neonatal diabetes mellitus is defined as hyperglycemia detected in the first month of life of more than 2 weeks' duration, requiring insulin treatment. It is extremely uncommon (1/500,000 neonates) and is permanent in only 30% of cases. Several hypotheses concerning its etiology have been postulated, such as pancreatic immaturity, paternal uniparental isidisomy of chromosome 6, and the existence of a gene located in the 6 q 22-23 chromosome region subjected to imprinting and exclusively of paternal expression. The management of these patients is usually difficult. These neonates are underweight for their gestational age, and neither anti-insulin antibodies nor anti-islets are detected. We studied a neonate hospitalized because of low weight for his gestational age with dimorphic features and hyperglycemia since the 17 th day of life. Clinical and anatomical follow-up has been periodically performed to the present date. The child presents permanent neonatal diabetes with negative antibodies. Although various insulin patterns have been used since the onset of the syndrome, management remains difficult. The child presents hypothyroidism, bilateral neurosensory deafness, bilateral congenital cataract, myopia, dimorphic features, congenital stridor and slow weight-stature curve. The results of muscle biopsy and metabolic studies were normal. Wolfram's syndrome and mitochondrial diabetes were ruled out. This is an exceptional case of permanent neonatal diabetes associated with other malformations corresponding to no known syndromic patterns.

Diabetes neonatal permanente asociada ahipotiroidismo, sordera y rasgos dismórficos / Permanent neonatal diabetes associated with other anomalies

La diabetes mellitus neonatal se define como una hiperglucemia detectada durante el primer mes de vida, de más de 2 semanas de duración, que precisa tratamiento con insulina. Es muy rara (1/500.000 recién nacidos) y sólo el 30% de los casos es permanente. Se han postulado varias hipótesis sobre su etiología, tales como inmadurez pancreática, isodisomía del cromosoma 6 paterno o la existencia de un gen localizado en la región cromosómica 6 q 22-23 sometido a impregnación y de expresión exclusivamente paterna. Se caracterizan por ser pacientes de difícil tratamiento, bajo peso para su edad gestacional y no se detectan anticuerpos antiinsulina ni antiislotes. Se ha estudiado un recién nacido ingresado por bajo peso para la edad gestacional con rasgos dismórficos e hiperglucemia desde el día 17 de vida. Se realiza el seguimiento clínico y analítico periódico hasta la actualidad, en el que se ha observado se trata de una diabetes neonatal permanente con anticuerpos negativos, y de difícil tratamiento a pesar de utilizar diversas pautas insulínicas desde el inicio del cuadro, hipotiroidismo, sordera neurosensorial bilateral, catarata congénita bilateral, miopía, rasgos dismórficos, estridor congénito y curva ponderostatural lenta. El estudio de biopsia muscular y metabólico fue normal. Se descartó un síndrome de Wolfram y una diabetes mitocondrial. Se trata de un caso excepcional de diabetes neonatal permanente asociado a otras malformaciones no encuadrable dentro de un patrón sindrómico conocido (AU)

Cultures of human osteoblastic cells from dialysis patients: influence of bone turnover rate on in vitro selection of interleukin-6 and osteoblastic cell makers.

The factors contributing to renal osteodystrophy are still incompletely characterized. A variety of cytokines and growth factors appear to have ill-defined roles in this disease. Our aim is to compare osteoblastic cell growth and different osteoblastic markers in vitro with histomorphometric bone parameters and some serum bone-turnover markers in vivo in dialysis patients with either high- (HTBD) or low-turnover (LTBD) bone disease. Six patients were diagnosed to have LTBD, and another five patients, HTBD. Intact parathyroid hormone (PTH) and osteocalcin (OC) levels in serum were greater in patients with HTBD than in those with LTBD. Osteoblastic cells isolated from iliac crest biopsy specimens were grown in culture medium for different times up to 13 days. Osteoblastic cell growth (cell number and area under the cell growth curve) was greater in patients with HTBD than in those with LTBD. Static and dynamic bone formation parameters correlated with serum PTH levels. No correlation was found between PTH and osteoblastic cell proliferation. OC, C-terminal type I procollagen, and alkaline phosphatase osteoblastic secretion in vitro were similar in the HTBD and LTBD groups. However, interleukin-6 (IL-6) secretion was greater in cells isolated from patients with LTBD. Our results indicate that osteoblastic cell growth and osteoblastic IL-6 secretion are related to bone turnover in patients with osteodystrophy. Our findings support the hypothesis that factors other than PTH level might have an important role in affecting osteoblastic function in renal osteodystrophy.

Primary giant cell tumor of soft tissues similar to bone giant cell tumor: A case report and literature review.

In this report we describe a primary giant cell tumor (GCT) of soft tissues located in the left dorsal wrist of a 52-year-old man. Plain radiographs did not reveal any lesion in his carpal or hand bones. Although the tumor was clinically considered a ganglion initially, the microscopic features were identical to those found in classic GCT of bone. Light microscopy showed a lesion composed of a homogeneously mixed proliferation of spindle and polygonal mononucleated stromal cells and evenly distributed multinucleated, osteoclast-like giant cells. Whereas most bone tumors have an extraosseous counterpart, only 13 cases of GCT in soft tissues had been published until 1998. Moreover, 64 new cases have been reported in three series. Nevertheless, most major reviews and textbooks do not consider this tumor as a specific entity and regard it as a low grade variant of malignant GCT of soft tissue. We describe the clinical, histologic, and immunohistochemical features of this rare benign neoplasm emphasizing the differential diagnosis with its malignant soft tissue counterpart, an ominous tumor.

Periosteal glomus tumor of the femur: a case report.

Osseous abnormalities produced by glomus tumors located in soft tissues of the periungual region have been described. More rare is the location of a glomus tumor within bone, which usually is located in the phalanx of the fingers. However, to the authors' knowledge, there is no previous description of a glomus tumor located in a periosteal location of a long bone. A 50-year-old man with a glomus tumor in a periosteal location of the lower metaphysis of the femur without neoplastic erosion of the cortical surface is reported. Magnetic resonance imaging and intraoperative ultrasonography were needed to locate the lesion.

Parathormone secretion in peritoneal dialysis patients with adynamic bone disease.

The prevalence of low-turnover lesions in patients undergoing peritoneal dialysis (PD) is high. Our aims are to evaluate the prevalence of adynamic bone disease (ABD) in PD patients, analyze risk factors, and define the association of serum parathyroid hormone (PTH) levels measured under different plasma calcium concentrations with this lesion. Fifty-seven patients were studied by bone biopsy (BB). ABD was found in 63.2%, and 36.8% showed high-turnover bone disease (HTBD). Patients with HTBD had a lower prevalence of diabetes, younger age, lower accumulated oral calcium salt intake, and greater calcitriol doses, serum osteocalcin level, and ultrafiltration than patients with ABD. Both mean baseline PTH levels from the previous year and PTH level at time of BB were greater in patients with HTBD than those with ABD (357 +/- 267 pg/mL versus 89 +/- 67 pg/mL; 390 +/- 337 pg/mL versus 88 +/- 78 pg/mL, respectively; P < 0.05). However, the magnitude of the increase from baseline serum PTH levels in response to hypocalcemia was greater in patients with ABD than in those with HTBD (166.4% +/- 134% versus 83.5% +/- 73.6%; P < 0.05). We found that PTH levels less than 150 pg/mL in patients with ABD showed a sensitivity of 91. 6%, specificity of 95.2%, and positive predictive value (PPV) of 97%. In the HTBD group, PTH levels greater than 450 pg/mL had a specificity and PPV of 100%. Our data confirm that ABD is the most prevalent lesion in PD patients, and PTH secretion capacity is maintained in these patients. The definitive diagnosis and management strategies for many patients requires a BB, especially when HTBD is unlikely.

Primary epithelioid leiomyosarcoma of bone. Case report and literature review.

We describe an epithelioid leiomyosarcoma of bone located in the right knee of a 51-year-old woman. Plain radiograph and CT scan revealed a poorly defined lytic and destructive mass in the upper metaepiphyseal right tibia which involved surrounding soft tissues. The lesion was composed of proliferating monotonous round cells with a high mitotic activity with scanty intersecting spindle cell fascicles. Immunohistochemistry of both areas demonstrated a strong positivity for actin (HHF-35 and alpha-SMA) and vimentin, and negative reactions for desmin, keratin (AE1 AE3), epithelial membrane antigen, S-100 protein, factor VIII-related antigen, CD 31 and CD 34. Ultrastructural study confirmed a diagnosis of leiomyosarcoma. This is the first detailed description of the microscopic and radiological features of primary epithelioid leiomyosarcoma of bone.