Impact of type 1 diabetes mellitus and celiac disease on nutrition and quality of life.

OBJECTIVE: Type 1 diabetes mellitus (T1DM) and celiac disease (CD) are autoimmune diseases and have similar genetic patterns. T1DM treatment is based on diet, physical activity and insulin therapy, whereas CD depends on dietary changes with restriction of wheat, rye and barley. The aim of the study was to evaluate the quality of life (QoL) of individuals with the association of T1DM and CD, to characterize their nutritional status and to compare it with those with only one disease and healthier controls. SUBJECTS/METHODS: Sixty patients controlled by sex, age and body mass index (BMI) were stratified by previous diagnosis in: T1DM and CD (DMCD group); T1DM (DM group); CD (CD group); or healthy participants (HC). The SF-36 questionnaire was applied to assess psychological well being and results were compared with glycemic control and presence of complications related to diabetes, adhesion to gluten-free diet (GFD). Nutritional status and body mass composition were determined by BMI, waist circumference, bioimpedance, general laboratory tests and whole-body densitometry. RESULTS: The time of diagnosis of T1DM was similar between DMCD and DM groups; however, the duration of CD was significantly higher in the CD group compared with DMCD. The SF-36 analysis revealed statistically significant differences between DM and HC groups in two domains: general health (P=0.042) and energy/vitality (P=0.012). QoL was also correlated with compliance to a GFD, and scores were similar in both groups: DMCD and CD. Forty percent of individuals in the CD group had visceral fat area above 100 cm2, as opposed to 20% in the other groups. CONCLUSIONS: Individuals of DMCD group had similar scores to DM, CD and HC on QoL, as well as on their nutritional status and bone metabolism. Thereby, we should conclude that the association of T1DM and CD did not deteriorate their health status.

Vitiligo y temperamento / Vitiligo and tempetament

A la fecha no existe ningún trabajo que explore la relación entre vitíligo y temperamento, salvo algunos que intentan buscar posibles causas. Menos aún trabajos desde una perspectiva del desarrollo que indaguen diversos factores psicobiológicos asociados a la génesis de este compromiso. Objetivo: Estudiar el temperamento, carácter y el respectivo desarrollo de pacientes con vitíligo, además de eventos vitales y calidad de vida. Método: Se analizaron 21 pacientes con diagnóstico de vitiligo infantil (G0), comparándolos con dos grupos controles,(G1) 14 hermanos sanos y (G2) 21 escolares sanos. Se utilizaron los siguientes instrumentos: 1.- JTCI ( Junior Temperament and Character Inventory) 2.- EDC ( Encuesta de Desarrollo Cualitativo) 3.- LEC ( Life Event Checklist) 4.- CDLQI ( Children’s Life Quality Index ). Resultados: Mediante el JTCI se encontró una frecuencia estadísticamente signifi cativa de dimensión Evitación del Daño (ED) en G0 vs G2 (13.6 v/s 10.6, p=0.037). En la EDC encontramos una mayor frecuencia de temor frente a extraños (71.4 por ciento v/s 35.7 por ciento) y una respuesta predominante de retraimiento y temor frente a cambios emocionales en figuras de apego (79,8 por ciento v/s 8,3 por ciento) G0 vs G1. En la LEC se encontró una relación en los eventos vitales del tipo situaciones de distanciamiento de figuras significativas. Conclusión: Al encontrar mayor frecuencia de ED en los niños con vitíligo más el resultado en la EDC, se corrobora la impresión clínica anterior, que estos niños tienen una especial sensibilidad que se asociaría a la génesis de este cuadro. Se inicia así, una línea de investigación, la perspectiva del desarrollo, para entender estos cuadros psico-somáticos de manera más integrada.

There is a lack for studies exploring the relationship between vitiligo and temperament, except those searching for possible causes. Yet, no study from a developmental view investigates different psychobiological factors related to the origin of this condition. Objective: to study the temperament, character and the respective development of vitiligo patients, the vital events and the life quality. Method: 21 patients with a diagnosis of childhood vitiligo (G0) were analyzed and compared to two control groups, 14 healthy brothers or sisters (G1) and 21 healthy school-aged children (G2). Instruments: 1. Junior Temperament and Character Inventory (JTCI), 2. Qualitative Psychosocial Development Survey (QPDS), 3. Life Event Checklist (LEC), 4.Children`s life quality index (CDLQI). Results: the JTCI revealed a significant statistics association between the harm avoidance dimension in the G0 group vs G2 (13.6 vs 10.6, p=0.037). The QPDS showed a higher frequency in fear to strangers(71.4 percent vs 35.7 percent) and a significant responsive fear- withdrawn pattern to the emotional variations of the attachment figure (G0 vs G1: 79.8 percent vs 8.3 percent).The LEC revealed an association between life events and …. Situations. Conclusions: the present study revealed a higher frequency for harm avoidance at the vitiligo group and by considering the QPDS results, the clinical impression was corroborate by suggesting the special sensitivity of this group of children in the origin of this pathology. An investigation line may start with a developmental and integrated view for the understanding of psychosomatic disorders.

Algunos aspectos del manejo del paciente quemado en un servicio de cirugía infantil: A propósito de 47 pacientes pediátricos / Some aspects burned patient management in a department of surgery child: About 47 pediatric patients

Si bien el manejo inicial de los niños quemados es fundamental para el resultado exitoso del tratamiento de esta cohorte de pacientes, el aspecto quirúrgico en el siguiente nivel tiene una importancia similar. En este estudio se presenta un análisis epidemiológico de 47 pacientes, tratados en nuestro Servicio en un período de dos años. Se esboza un análisis de los datos estadísticos obtenidos, se sacan conclusiones y se elaboran normas de profilaxis.

While the initial management of burned children is fundamental to the successful outcome of treatment of this cohort of patients, surgical appearance at the next level has an equal importance. This study provides an epidemiological analysis of 47 patients treated in our department in a period of two years, outlined an analysis of statistical data obtained, conclusions drawn and develop standards for prophylaxis.

Human herpes simplex virus infections: epidemiology, pathogenesis, symptomatology, diagnosis, and managemenT

Eight of the more than 80 known herpesviruses are human pathogens. Human herpes simplex virus (HSV) is a contagious infection with a large reservoir in the general population. It has a potential for significant complications in the immunocompromised host. In addition, psychological distress caused by the negative stigma associated with genital herpes and visible facial lesions in those experiencing frequent outbreaks renders it a challenging clinical dilemma. This article reviews the epidemiology, pathogenesis, and diagnostic features of HSV infections, providing the clinician with an up-to-date understanding of the available management strategies for mucocutaneous HSV-induced disease.

Sentinel node in breast cancer: experience with periareolar 99mTc-Dextran / Ganglio centinela en cancer de mama: experiencia con 99mTc-Dextran periareolar

Se presentan los resultados de la biopsia de ganglio centinela en 77 mujeres portadoras de cáncer ductal in situ (CDIS) multifocal o cáncer de mama T1-2 , todas con axila clínicamente negativa, utilizando inyección periareolar intradérmica de 99mTc-Dextran, linfocintigrafía y detección radioisotópica intraoperatoria. En 72/77 pacientes además se usó colorante azul de Isosulfan. Se obtuvo al menos un ganglio centinela en la totalidad de las pacientes. Se encontró 1 o 2 ganglios metastásicos en 14 pacientes. En 13 de ellas el ganglio centinela estaba comprometido y en 6/10 con disección axilar (DA) el resto de los ganglios estaban negativos.

Sentinel lymph node biopsy (SLNB) was performed in 77 consecutive women with clinically node-negative T1 and T2 breast cancer or multifocal ductal carcinoma in situ (DCIS). They had periareolar subdermal injection of 99mTc-Dextran, preoperative lymphoscintigraphy and intraoperative handheld probe detection. Isosulfan blue dye was also used in 72/77 patients. At least one sentinel node was found at surgery for all patients. In 14 patients one or two metastatic nodes were found, 13 of them in the sentinel node(s). Axillary lymph node dissection was otherwise negative in 6/10 positive SLNB patients.

Utilidad del PET 18F-fluordeoxiglucosa en melanoma maligno / 18F-fluorodeoxyglucose positron emission tomography in patients with malignant melanoma

Está establecida en la literatura la utilidad de la tomografía por emisión de positrones (PET) con 18F-flúordeoxiglucosa (FDG) en la etapificación, reetapificación y seguimiento del melanoma maligno. Objetivo: Evaluar los resultados del PET FDG en melanoma maligno en nuestro centro. Material y Método: Entre febrero 2003 y julio 2004, se estudiaron 33 pacientes (edad 49±14 años, 52% sexo masculino) referidos para etapificación y reetapificación de melanoma maligno. El examen fue realizado en equipo de alta resolución Siemens Ecat Exact HR+ con dosis de 13±3 mCi de FDG y glicemias en ayunas preinyección de 96±16 mg/dL. Se adquirieron imágenes de cuerpo entero, incluyendo cabeza y extremidades inferiores. El informe se basó en el análisis visual e índice cuantitativo de captación (SUV). Se comparó con otros estudios de imágenes e histología cuando estaban disponibles y se realizó seguimiento clínico. Resultados: Nueve pacientes fueron derivados para etapificación y 24 para reetapificación. En 29 casos, la localización inicial del primario era conocida y en 4 la enfermedad se diagnosticó por la presencia de metástasis ganglionares. Respecto de la localización tumoral, 6 fueron en cabeza y cuello (2 metástasis ganglionares), 5 en coroides, 5 en tronco, 5 en extremidades superiores, 11 en extremidades inferiores y 1 en mucosa (rectal). En 13 pacientes, el PET fue positivo para presencia de actividad tumoral hipermetabólica, 7 en ubicación próxima al primario y 6 alejados de éste, correspondiendo uno de estos últimos a un segundo primario (carcinoma rectal confirmado con histología). De este grupo, en 11 pacientes se confirmó tumor: en 7 hubo confirmación histológica postcirugía y en los restantes, hubo concordancia con imágenes anatómicas y clínica (1 paciente falleció); además, el PET demostró lesiones no sospechadas por otros estudios, las que no han sido confirmadas con histología. Dos fueron falsos positivos, uno por hematoma y otro por ...

Background: The usefulness of positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) for the staging and follow up of malignant melanoma, is well established. Aim: To assess the results of PET FDG in patients with malignant melanoma. Patients and Methods: Thirty three patients with malignant melanoma (aged 49 ± 14 years, 17 males), referred for staging and restaging, were studied. The tomography was performed using a Siemens Ecat Exat high resolution equipment. The dose of FDG was 13 ± 3 mCi and the blood glucose levels prior to injection were 96 ± 16 mg/dl. Whole body images, including the head and legs were acquired. The report was based on the visual analysis and standardized uptake value (SUV). Results: Nine patients were referred for staging and 24 for restaging. The location of the primary tumor was known in 29 cases and in four, the disease was diagnosed due to the presence of lymph node metastases. The tumor was located in the head and neck in six patients (including two lymph node metastases), in the choroid in five, in the trunk in five, in the superior limbs in five, in the inferior limbs in 11 and in the rectal mucosa in one. In 13 patients, PET was positive for the presence of hypermetabolic tumor activity. In seven, the location was near the primary tumor and in six, it was distant. In seven patients the presence of tumor was confirmed by surgery and in four, there was concordance between the anatomical imaging and clinical outcome. Two false positive images were detected, one hematoma and one due to the presence of lymph node inflammation. In 16 cases, PET was negative and in four it was not conclusive, all these patients do not have evidence of tumor in the clinical follow up. The primary location of the tumor was not identified in patients referred for lymph node metastases. Conclusions: FDG PET is useful for the staging and re staging of patients with malignant melanoma.

Retropharyngeal abscess: epiglottitis of the new millennium.

From 1993 through 1999, 26 children with retropharyngeal abscess and 2 children with acute epiglottitis were cared for by pediatric otolaryngologists in northern Virginia. Fever, sore throat, dysphagia, refusal to swallow, dysphonia, drooling, and neck extension are common presenting signs and symptoms in acute epiglottitis and in retropharyngeal abscess. Contrast-enhanced computed tomography of the oropharynx was performed in all cases and was the most helpful diagnostic test.

Nutritional rickets in African American breast-fed infants.

OBJECTIVE: To analyze the characteristics of infants and children diagnosed with nutritional rickets at two medical centers in North Carolina in the 1990s. STUDY DESIGN: The physical and radiographic findings, calcium, phosphorus, alkaline phosphatase, and 25-hydroxyvitamin D levels of infants and children diagnosed with nutritional rickets at two medical centers were reviewed. Breast-feeding data were obtained from the North Carolina Women, Infants and Children Program (WIC). RESULTS: Thirty patients with nutritional rickets were first seen between 1990 and June of 1999. Over half of the cases occurred in 1998 and the first half of 1999. All patients were African American children who were breast fed without receiving supplemental vitamin D. The average duration of breast-feeding was 12.5 months. The age at diagnosis was 5 to 25 months, with a median age of 15.5 months. Growth failure was common: length was <5th percentile in 65% of cases, and weight was <5th percentile in 43%. CONCLUSION: Factors that may have contributed to the increase in referrals of children with nutritional rickets include more African American women breast-feeding, fewer infants receiving vitamin D supplements, and mothers and children exposed to less sunlight. We recommend that all dark-skinned breast-fed infants and children receive vitamin D supplementation.

The prevalence of celiac disease in at-risk groups of children in the United States.

OBJECTIVE: In contrast to its prevalence in Europe, celiac disease (CD) is considered rare in the United States. We aimed to determine the prevalence of CD in children presenting with symptoms or conditions associated with CD. STUDY DESIGN: Individuals aged 6 months to 20 years were screened for IgG and IgA antigliadin (AGA-IgG and AGA-IgA) and antiendomysium (EMA) antibodies. Those with only elevated AGA-IgG were screened for selective IgA deficiency. Patients with elevated EMA, or AGA-IgG elevation and selective IgA deficiency, were advised to undergo small intestinal biopsy. RESULTS: A total of 1200 individuals were studied; 34 were EMA positive-26 (19 EMA positive) consented to biopsy and 21 had CD, giving a prevalence of 1 in 57 (21/1200). Including the 15 EMA positive patients who refused a biopsy, the prevalence of CD in this study could be as high as 1 in 33 (36/1200). CONCLUSIONS: CD is not rare in the United States and may be as common as in Europe. AGA and EMA are useful for identifying patients who should undergo a small intestinal biopsy.

Familial medullary thyroid carcinoma: presymptomatic diagnosis and management in children.

Two kindreds with familial medullary thyroid carcinoma (MTC) are described in which affected family members had variable clinical and pathologic manifestations. Genetic testing in 2 children from one kindred revealed a mutation in exon 10, codon 618 (TGC to AGC) in the extracellular cysteine-rich region of the RET gene. In this kindred an 11-year-old had microscopic evidence of MTC; however, a 17-year-old had no evidence of pathology on thyroidectomy. In a second kindred a rare mutation in exon 14, codon 804 (GTG to TTG) of the intracellular tyrosine kinase region of the RET gene was detected. In this kindred MTC has occurred in the 4th to 5th decades of life, with a clinical spectrum in mutation-positive family members ranging from no disease and C-cell hyperplasia to carcinoma with lymph node metastasis; a 7-year-old with the mutation and a normal response to provocative testing was also identified. Management recommendations in children from families with clearly defined familial MTC may be individualized to reflect emerging genotype-phenotype correlations.

Dose-related efficacy of budesonide administered via a dry powder inhaler in the treatment of children with moderate to severe persistent asthma.

OBJECTIVE: To determine the efficacy and safety of budesonide delivered by an inhalation-driven dry powder inhaler (Turbuhaler) in children with moderate to severe persistent asthma. STUDY DESIGN: In our randomized, double-blind, placebo-controlled, parallel-group, multicenter study, a total of 404 children with asthma, who were aged 6 to 18 years and who had been receiving inhaled glucocorticosteroid therapy, were randomly assigned to receive either 100, 200, or 400 micrograms of budesonide or placebo twice daily for 12 weeks. At baseline, mean forced expiratory volume in 1 second (FEV1) was 74.6% (range, 30.7% to 123.3%) of the predicted normal value. RESULTS: Patients in each of the three budesonide treatment groups showed significant dose-related improvements in lung function (morning peak expiratory flow and FEV1), in asthma symptoms, and with a significant decrease in inhaled beta 2-agonist use in comparison with placebo. Improvements were evident within 2 weeks and were maintained throughout the 12 weeks. Budesonide treatment had no significant effect on hypothalamic-pituitary-adrenal axis function, and the incidence of reported adverse events was similar in all treatment groups. CONCLUSION: Budesonide administered via a dry powder inhaler provided dose-related improvements in lung function and clinical status and was well tolerated by children (6 to 18 years of age) with moderate to severe persistent asthma.

Verrucous carcinoma of the skin and mucosa

The concept of verrucous carcinoma as a clinicopathologic variant of squamous cell carcinoma is worthy of recognition. It is known by a confusing array of names, such as Ackerman's tumor, Buschke-Loewenstein tumor, florid oral papillomatosis, epithelioma cuniculatum, carcinoma cuniculatum, and cutis papillomatosis carcinoides of Gottron. Its apparent clinical benignity may lead to lengthy periods of misdiagnosis, during which it is likely not to spread to distant lymph nodes, but rather to destroy a nose, mandible, or penis as it slowly but relentlessly extends into underlying tissue. Morphologically warty or verrucous, its relatively bland histologic features are often more suggestive of a verruca vulgaris or pseudoepitheliomatous hyperplasia than of squamous cell carcinoma to those unfamiliar with the diagnosis. Alternatively, when it extends into underlying tissues, it may be mistaken histologically for a benign adnexal tumor or even an epidermoid cyst. Therapy may also be challenging because recurrences are common and concerns about potential anaplastic transformation after radiotherapy are often expressed.

Tracheal tube leak test--is there inter-observer agreement?

Although the leak test is recommended as a method of assessing the appropriate size of uncuffed endotracheal tubes for use in children, the reproducibility of this test has not been validated. Patients from newborn to ten years of age requiring tracheal intubation for elective surgery were studied. The endotracheal tube size was calculated using the formula: (age + 16) divided by 4 for patients > or = two years of age and at the discretion of the attending anaesthetist for patients < two years of age. After the induction of anaesthesia and administration of a nondepolarizing muscle relaxant, the patient's trachea was intubated and mid-tracheal placement was confirmed. Two of the three staff anaesthetists participating in the study assessed the leak pressure consecutively. Each participant performed a single leak determination. The leak pressure was determined as follows: the patient was supine with the head in a neutral position, fresh gas flowed into the breathing circuit at 5 L.min-1, a stethoscope was placed on the skin over the larynx and the pressure relief valve was completely closed. Pressure slowly increased in the breathing circuit until an audible leak occurred around the tracheal tube. The inter-observer difference was calculated in 212 patients. The absolute value of the difference between that of two observers increased as the mean leak pressure increased. However, the variation between observers expressed as a percent of the absolute measurement remained constant. An average variance in measurement of 38% was found at both low and high leak pressures. In conclusion, we found considerable variation between two experienced observers in assessing leak pressures.(ABSTRACT TRUNCATED AT 250 WORDS)