In biliary atresia (BA), efforts to prevent premature liver transplantation (LT) are aimed at early diagnosis, timing of Kasai-portoenterostomy (KPE), and centralization of care. This report presents the clinical picture, treatment strategies, and outcomes of BA patients with no previous treatment. A retrospective cohort study (Jan/2001 to Jan/2021) was conducted to evaluate the outcome of patients with BA referred to a single team. Study groups were: 1) Kasai-only group (K-only) n=9), 2) LT-only group (n=7), and 3) Kasai+LT group (K+LT) (n=23). Survival with native liver and overall survival were 22.9 and 94.8%, respectively, at 120 months of follow-up. There was no difference in age at KPE in the K-only group (46.8±21.8 days) vs K+LT (52.1±22 days), P=0.4. Ten (25.6%) patients were babies conceived through in vitro fertilization (IVF). Four IVF patients (40%) presented associated congenital heart disease vs 5 patients (17%) in the remaining group (P=0.14). Two of the IVF patients were premature (<37 weeks). Median maternal age at birth was 35 years (33 to 41 years). Excellent patient survival is expected for patients with BA with the available treatment strategies. IVF+BA was an unexpected prevalent association in this cohort, and further studies are required to better understand these findings.

The intracranial compliance in type 2 diabetes mellitus (T2DM) patients and the association with cardiovascular autonomic control have not been fully elucidated. The aim of this study was to assess intracranial compliance using the noninvasive intracranial pressure (niICP) and the monitoring of waveform peaks (P1, P2, and P3) and the relationship with cardiovascular autonomic control in T2DM patients. Thirty-two men aged 40-60 years without cardiovascular autonomic neuropathy (CAN) were studied: T2DMG (n=16) and control group CG (n=16). The niICP was evaluated by a noninvasive extracranial sensor placed on the scalp. Cardiovascular autonomic control was evaluated by indices of the baroreflex sensitivity (BRS), from temporal series of R-R intervals of electrocardiogram and systolic arterial pressure, during supine and orthostatic positions. The participants remained in the supine position for 15 min and then 15 min more in orthostatism. T2DMG presented a decrease of the P2/P1 ratio during the orthostatic position (P<0.001). There was a negative moderate correlation between the P2 peak with cardiovascular coupling (K2HP-SAPLF) in supine (r=-0.612, P=0.011) and orthostatic (r=-0.568, P=0.020) positions in T2DMG. We concluded that T2DM patients without CAN and cardiovascular complications presented intracranial compliance similar to healthy subjects. Despite preserved intracranial adjustments, T2DM patients had a response of greater magnitude in orthostatism. In addition, the decoupling between the heart period and blood pressure signal oscillations in low frequency appeared to be related to the worsening of intracranial compliance due to the increased P2 peak.

Frailty is related to a decrease in the physiological reserves, which causes difficulties in maintaining homeostasis. An example of physiological mechanisms for cardiovascular homeostasis is the baroreflex. The aim of this study was to compare baroreflex among frail, prefrail, and nonfrail individuals, in supine and orthostatic positions. Community-dwelling older adults were evaluated and categorized into frail, prefrail, or nonfrail groups, according to frailty phenotype. The RR interval (RRi) and systolic blood pressure (SBP) series were recorded for 15 min in the supine and 15 min in the orthostatic positions. Mean and variance of RRi and SBP, and baroreflex evaluated by phase, gain (α), and coherence (K2) were determined. A two-way repeated measures ANOVA, with Tukey's post hoc, was applied for group, position, and their interaction effects. The significance level established was 5%. Prefrail and frail participants did not present a significant decrease in mean values of RRi after postural challenge (893.43 to 834.20 ms and 925.99 to 857.98 ms, respectively). Frail participants showed a reduction in RRi variance in supine to orthostatic (852.04 to 232.37 ms2). Prefrail and frail participants showed a decrease in K2 after postural change (0.69 to 0.52 and 0.54 to 0.34, respectively). Frail participants exhibited lower values of K2 (0.34) compared to nonfrail and prefrail participants (0.61 and 0.52, respectively). Baroreflex indicated the presence of decoupling between heart period and SBP in frail and prefrail. Thus, reduced K2 might be a marker of the frailty process.

Maple syrup urine disease (MSUD) is an autosomal recessive disease associated with high levels of branched-chain amino acids. Children with MSUD can present severe neurological damage, but liver transplantation (LT) allows the patient to resume a normal diet and avoid further neurological damage. The use of living related donors has been controversial because parents are obligatory heterozygotes. We report a case of a 2-year-old child with MSUD who underwent a living donor LT. The donor was the patient's mother, and his liver was then used as a domino graft. The postoperative course was uneventful in all three subjects. DNA analysis performed after the transplantation (sequencing of the coding regions of BCKDHA, BCKDHB, andDBT genes) showed that the MSUD patient was heterozygous for a pathogenic mutation in the BCKDHB gene. This mutation was not found in his mother, who is an obligatory carrier for MSUD according to the family history and, as expected, presented both normal clinical phenotype and levels of branched-chain amino acids. In conclusion, our data suggest that the use of a related donor in LT for MSUD was effective, and the liver of the MSUD patient was successfully used in domino transplantation. Routine donor genotyping may not be feasible, because the test is not widely available, and, most importantly, the disease is associated with both the presence of allelic and locus heterogeneity. Further studies with this population of patients are required to expand the use of related donors in MSUD.

The objective of this study was to use linear and non-linear methods to investigate cardiac autonomic modulation in healthy elderly men and women in response to a postural change from the supine to the standing position. Fourteen men (66.1 ± 3.5 years) and 10 women (65.3 ± 3.3 years) were evaluated. Beat-to-beat heart rate was recorded in the supine and standing positions. Heart rate variability was studied by spectral analysis, including both low (LFnu-cardiac sympathetic modulation (CSM) indicator) and high (HFnu-cardiac vagal modulation (CVM) indicator) frequencies in normalized units as well as the low frequency/high frequency (LF/HF) ratio. Symbolic analysis was performed using the following indexes: 0V% (CSM indicator), 1V% (CSM and CVM indicators), 2LV% (predominantly CVM indicator) and 2ULV% (CVM indicator). Shannon entropy was also calculated. Men presented higher LFnu and LF/HF ratio and lower HFnu and 1V% symbolic index (57.56, 4.14, 40.53, 45.96, respectively) than women (24.60, 0.45, 72.47, 52.69, respectively) in the supine position. Shannon entropy was higher among men (3.53) than among women (3.33) in the standing position, and also increased according to postural change in men (3.25; 3.53). During postural change, the LFnu (24.60; 49.85) and LF/HF ratio (0.45; 1.72) increased, with a concomitant decrease in HFnu (72.47; 47.56) and 2LV% (14.10; 6.95) in women. Women presented increased CSM in response to postural change and had higher CVM and lower CSM than men in the supine position. In conclusion, women in the age range studied presented a more appropriate response to a postural change than men, suggesting that cardiac autonomic modulation may be better preserved in women than in men.

The exact solution of the inhomogeneous wave equation in one dimension, when the square of the velocity is a linear function of the position, can be written in terms of Bessel functions of the first kind. We use this solution as the zero order approximation for a perturbation expansion and apply it to the case when the square of the velocity can be written as a polynomial in the position. The first and second order perturbation terms, corresponding to quadratic and cubic terms for the square of the velocity, are obtained. A closed formula for the n-order correction in terms of integrals of the Bessel functions of the first kind was also explicitly obtained, this expression can be solved analytically for the first and second order corrections and numerically for higher terms

La solución exacta de la ecuación de onda inhomogénea en una dimensión, cuando el cuadrado de la velocidad es una función lineal de la posición, puede escribirse en términos de las funciones Bessel de primera especie. Usamos esta solución como la aproximación de orden cero de un desarrollo perturbativo y lo aplicamos al caso cuando el cuadrado de la velocidad puede escribirse como un polinomio de grado n. Obtuvimos explícitamente las perturbaciones de primer y segundo orden correspondientes a los términos cuadráticos y cúbicos para el cuadrado de la velocidad. También se encontró una expresión cerrada para la corrección a orden n en terminos de integrales de funciones Bessel de primera especie; esta puede resolverse analíticamente para el primer y segundo orden y numéricamente para ordenes superiores

A case report is presented of a patient with a lip ulcer that led to a primary diagnosis of infection through Treponema pallidum and to a secondary diagnosis of HIV seropositivity. The diagnosis was made by serology and the antibiotic treatment was effective for the syphilis. We conclude that when faced by a lesion of the lip that has an ulcerous appearance, a differential diagnosis should be contemplated that includes a syphilis chancre and, if this is confirmed, the necessary complementary test should be carried out in order to rule out concomitant HIV infection.

Se presenta el caso de un paciente con úlcera labial cuya apariencia clínica simula un carcinoma escamoso. Sin embargo, una correcta historia clínica y la realización de pruebas complementarias conducen al diagnóstico primario de infección por Treponema pallidum y secundario de seropositividad para VIH. Tras el diagnóstico serológico el tratamiento antibiótico es efectivo para la sífilis. Concluimos que ante una lesión labial de aspecto ulceroso debe contemplarse un diagnóstico diferencial que incluya el chancro sifilítico y realizar, en el caso de que se confirme, las pruebas complementarias necesarias para descartar infección concomitante por VIH.

The basal cell adenoma is a specific type of adenoma, with a uniform, monomorphous histologic appearance that is dominated by basaloid cells and that does not have the myxochondroid tissue characteristic of mixed tumors. It may be divided on the basis of its morphologic pattern into four subtypes: solid, tubular, trabecular and membranous. We report four cases of basal cell adenoma subdivided as follows: one solid, one trabecular and two membranous subtypes. In all cases a conservative superficial parotidectomy was the treatment. Morphologic identification of the specific subtype is for descriptive purposes, except in the case of the membranous type, due to its tendency to be multifocal, its high recurrence rate, its occasional malignant transformation and its possible association in about onethird of the reported cases with dermal cylindromas. A close followup and screening of skin lesions is suggested for these tumors.

El adenoma de células basales es un tipo específico de adenoma con una apariencia histológica uniforme y monomorfa, en el que predominan las células basaliodes sin el componente mixocondroide del tumor mixto. Atendiendo a su morfología pueden ser divididos en cuatro subtipos: sólido, tubular, trabecular y membranoso. Presentamos cuatro casos de adenoma de células basales localizados en glándula parótida: uno de tipo sólido, uno de tipo trabecular y dos de tipo membranoso, tratados mediante parotidectomía superficial conservadora en todos los casos. Esta división en distintos patrones morfológicos tiene una finalidad descriptiva, salvo en el subtipo membranoso por su mayor tendencia a la multifocalidad y a la recidiva, su ocasional transformación maligna, así como por su posible asociación en un tercio de los casos a tumores ecrinos dermales. Esto implica un seguimiento más estrecho y un despistaje de posibles lesiones cutáneas asociadas.

Condylar damage can produce temporomandibular joint ankylosis. If ankylosis ocurrs in early childhood can develope facial asymmetry or mandibular hypoplasia. In these cases, several therapeutic options have been used, but distraction osteogenesis offers a new excellent alternative because it allows elongation of soft tissues and can be performed in childhood. We present a bilateral temporomadibular joint ankylosis in a 9 years old girl, who was treated by means an arthroplasty and extraoral distraction devices, only for elongation of soft tissues and keeping space between skull base and mandibula to avoid reankylosis and to allow condylar remodelation.

Los traumatismos que afectan a los cóndilos mandibulares pueden ocasionar una anquilosis de la articulación temporomandibular que, si se produce en edad de crecimiento, puede dar lugar a una deformidad facial con asimetría o retrognatia. Entre los diferentes tratamientos utilizados, la distracción osteogénica ofrece unas ventajas, entre ellas la posibilidad de aplicación en la infancia y la elongación de tejidos blandos. Presentamos un caso de anquilosis temporomandibular bilateral en una niña de 9 años, que tratamos con distracción extraoral tras resección del bloque anquilótico, pero sólo con la finalidad de elongar los tejidos blandos y mantener el espacio en la nueva cavidad glenoidea para evitar la reanquilosis y favorecer la remodelación del cóndilo.

Os autores descrevem um aparelho destinado à medida contínua da água absorvida pela TFSA em condições de laboratório. O sorbímetro, cujo funcionamento obedece ao princípio dos vasos comunicantes, consta de um tubo em U cujos ramos verticais se ligam respectivamente à uma coluna de terra e à uma bureta de 100 ml. A bureta restabelece no tubo, a água absorvida pela terra. As leituras de bureta substituem as pesagens do método gravimétrico. O aparelho é recomendado para medidas a intervalos de tempo para os quais, o método usual é ineficiente.

This paper describes an apparatus designed to carry on the continuous measurement of the water absorption by soils under laboratory conditions. The apparatus was called "sorbímetro". The principle accordingly to the "sorbimetro" works is the same which governs the flow of water in the usual linked flasks. The apparatus has a U tube whose vertical arms are connected respectivelly, to a soil colunn and to a 100 ml burete; the burete keeps constant the volume of water in the U tube since the liquid is being absorbed by the soil. In the present method the readings on the burete substitute for the usual weighings. The method is recommended for measurements in time intervals in which the gravimetric method does not work too well.