Immature ovarian teratoma in a 20-year-old woman: A case report.

Immature ovarian teratomas are a rare subtype of germ cell tumours characterized by the presence of embryonic elements, particularly primitive neuroepithelium, and they typically affect young women. We report the case of a 20-year-old woman who presented with a growing abdominal mass that turned out to be a grade II immature teratoma after adnexectomy. This article reviews the clinical presentation, imaging features, and some of the main problems that arise in the management of immature ovarian teratomas.

[Recurrent acute fatty liver of pregnancy: About a case and a review of the literature]. / La stéatose hépatique aiguë gravidique récidivante : à propos d'un cas et une revue de la littérature.

Acute fatty liver of pregnancy (AFLP) is a specific but rare hepatopathy that can usually complicate the third trimester of pregnancy. It is potentially fatal for the mother and the fetus. To our knowledge, only eight cases of recurrence have been published, we report a new case. The first episode presented by our 23-year-old patient was suspected in front of a cutaneous-mucosal jaundice with vomiting occurring on pregnancy of 35weeks of gestation (WG). Hyperleucytosis, abnormalities of the hepatic balance, as well as a hypoglycemia were biological elements supporting the diagnostic beam. On the other hand, medical imaging could not bring a clear confirmation. The evolution was favorable after deferred delivery by caesarean section for pulmonary maturation. Three years later, she presented to the obstetrical emergency room at 36weeks and six days of gestation, with a clinical and biological picture almost similar to that of the first episode. A caesarean section was then indicated for suspicion of recurrence. The evolution is favorable for the mother and her children. The interest of the communication on the risk of recurrence, the clinical and biological monitoring in particular in the third trimester of the subsequent pregnancy are imperative, in order to improve the prognosis of this pathology.

World epidemiological meta-analyses of peste des petits ruminants (1997-2017).

Estimating the occurrence and distribution of infection and identification of risk factors remain key components in understanding the epizootiology and monitoring of peste des petits ruminants (PPR). This study was performed from 1997 to 2017, and included details of flocks with emergent infections, within-flock prevalence and risk association between PPR incidence and various flock management factors worldwide. In assessing the impact of PPR on livestock, outbreak incidence per country was used as an effective indicator of the intensity of the infection process. To decode the spatial and temporal dynamics of PPR outbreaks and clarify their relationship with geographical factors, systematic review and logistic regression analyses were conducted. The impact of climatic and socio-economic conditions on PPR was moderate and high, respectively. In the PPR risk analysis, infected PPR zones were 1.68 times more likely to spread the infection to goat farms than to sheep farms (relative risk: 1.69; odds ratio: 3.26). Moreover, during PPR occurrence, goats are more susceptible to infection than sheep. Through a regression model of outbreaks, a value of 960.67 outbreaks was calculated as the expected mean in 2018. The polynomial regression of PPR cases was followed by extrapolation (medium-sized smoothing of the three following points) to define the expected value in 2018. The probability of PPR could be effectively reduced by coordinating the work in disadvantaged countries with low-income farmers, and disease control must be prioritised to support alleviation of poverty, which has a negative impact on livestock production.

L'estimation de la fréquence et de la distribution de l'infection par le virus de la peste des petits ruminants (PPR) et la définition des facteurs de risque de PPR sont des éléments essentiels pour comprendre l'épizootiologie de cette maladie animale et en assurer le suivi. Les auteurs présentent les résultats d'une étude conduite de 1997 à 2017 pour caractériser les troupeaux enregistrant de nouveaux cas d'infection, déterminer la prévalence au sein des troupeaux et relever les éventuelles corrélations entre l'incidence de la PPR et un certain nombre de facteurs liés à la gestion des troupeaux dans le monde. L'incidence nationale des foyers, considérée comme un indicateur pertinent de l'intensité du processus infectieux, a été utilisée pour évaluer l'impact de la PPR sur les animaux d'élevage. Un examen systématique et des analyses de régression logistique ont permis de faire ressortir la dynamique spatiale et temporelle des foyers de PPR et d'élucider leur lien avec certains facteurs géographiques. Il est ainsi apparu que les conditions climatiques ont eu un impact modéré sur la PPR tandis que l'impact des conditions socioéconomiques était élevé. L'analyse des risques de PPR dans les zones affectées a fait apparaître un risque de propagation supérieur d'un ordre de grandeur de 1,68 dans les élevages caprins par rapport aux élevages ovins (risque relatif : 1,69 ; rapport de cotes ou odds ratio [OR] : 3,26). En outre, en cas d'émergence de la PPR, les caprins sont davantage sensibles au virus que les ovins. Un modèle de régression a permis de calculer une valeur attendue de 960,67 foyers en moyenne pour l'année 2018. Cette valeur attendue pour 2018 a été définie par extrapolation (lissage moyen des trois points suivants) de la régression polynomiale du nombre de cas de PPR. La probabilité de survenue de la PPR pourrait être efficacement réduite en coordonnant les activités en direction des éleveurs à faible revenus dans les pays défavorisés ; les activités de lutte contre la maladie doivent viser en priorité à réduire la pauvreté, qui a un impact négatif sur la production animale.

Para conocer la epizootiología de la peste de pequeños rumiantes (PPR) y hacer un seguimiento de la infección hay dos procesos que siguen siendo básicos: determinar la frecuencia de aparición y la distribución de la enfermedad; e identificar los factores de riesgo. Los autores presentan un estudio realizado entre 1997 y 2017 en el que se analizaron, a escala mundial, una serie de características de los rebaños con nuevos casos de infección, la prevalencia dentro de cada rebaño y la correlación de riesgo existente entre la incidencia de la PPR y diversos factores ligados a la gestión de los rebaños. Para evaluar el impacto de la PPR en el ganado se empleó la incidencia de brotes por país, considerada un indicador eficaz de la intensidad del proceso infeccioso. Gracias a un estudio sistemático y a varios análisis de regresión logística se pudo dilucidar la dinámica espacial y temporal de los brotes de PPR y esclarecer su relación con factores geográficos. Se observó así que las condiciones climáticas influían moderadamente en la PPR y que las condiciones socioeconómicas tenían una influencia muy marcada. El análisis del riesgo de PPR reveló que, en las zonas afectadas, la probabilidad de propagación a explotaciones caprinas era 1,68 veces mayor que la de propagación a explotaciones ovinas (riesgo relativo: 1,69; razón de probabilidades [OR]: 3,26). Además, en el curso de un episodio de PPR, las cabras son más sensibles a la infección que las ovejas. Aplicando a los brotes un modelo de regresión se obtuvo un valor de 960,67 brotes como promedio previsto de brotes para 2018, calculado por extrapolación (alisado de tamaño medio de los tres puntos siguientes) a partir de la regresión polinómica de casos de PPR. Sería posible reducir eficazmente la probabilidad de PPR coordinando las actividades dirigidas a los productores de bajo nivel de renta en los países desfavorecidos, además de lo cual es imperativo otorgar prioridad al control de la enfermedad para secundar la reducción de la pobreza, sabiendo que esta repercute negativamente en la producción ganadera.

Auditory system dysfunction due to infantile thiamine deficiency: long-term auditory sequelae.

Eleven infants who were fed a thiamine-deficient formula for a mean of 3 months were evaluated for immediate and long-term auditory abnormalities. At presentation, 8 infants had auditory neuropathy spectrum disorder (ANSD), which resolved with supplementary thiamine in 5 children, was permanent in 2 children, and deteriorated in 1 patient who died at the age of 7 years. An additional patient had an auditory pattern corresponding to that of auditory neuropathy of brain stem origin. The 2 remaining patients had unilateral cochlear hearing loss. Six to 8 years later, all patients with transient ANSD had normal audiograms, 2 patients had unilateral cochlear hearing loss, and the rest had neural hearing loss. All survivors had a language developmental delay and impaired speech intelligibility of varying degrees, especially in the presence of background noise. Thiamine is crucial for normal auditory development and function, and its deficiency may be considered an acquired metabolic cause of ANSD in infants.

Investigation of the microstructure of milk protein concentrate powders during rehydration: alterations during storage.

The aim of this work was to use scanning electron microscopy to investigate the microstructure of rehydrated milk protein concentrate powder (MPC) particles. A sample preparation method for scanning electron microscopy analysis of rehydrated MPC particles is described and used to characterize the time course of dissolution and the effects of prior storage on the dissolution process. The results show that a combination of different types of interactions (e.g., bridges, direct contact) between casein micelles results in a porous, gel-like structure that restrains the dispersion of individual micelles into the surrounding liquid phase without preventing water penetration and solubilization of nonmicellar components. During storage of the powder, increased interactions occur between and within micelles, leading to compaction of micelles and the formation of a monolayer skin of casein micelles packed close together, the combination of which are proposed to be responsible for the slow dissolution of stored MPC powders.

Epilepsy in children with infantile thiamine deficiency.

OBJECTIVE: To report the follow-up findings of 7 children with severe epilepsy as a result of thiamine deficiency in infancy caused by a defective soy-based formula. METHODS: The medical records of 7 children aged 5-6 years with thiamine deficiency in infancy who developed epilepsy were reviewed and their clinical data, EEG tracings, and neuroimaging results were recorded. The clinical course and present outcome of these children, now 5 years after exposure to thiamine deficiency, are described. RESULTS: All infants displayed seizures upon presentation, either tonic, myoclonic, or focal. Six infants had an EEG recording at this stage and all showed slow background. Five of them had no epileptic activity and only 1 displayed focal activity. Following a seizure-free period of 1-9 months, the seizures recurred, and all 7 children displayed either myoclonic or complex partial seizures. Multifocal or generalized spike wave complexes were recorded on the EEGs of all 7 patients, and the tracings of 3 children evolved into hypsarrhythmia. The seizures were refractory to most antiepileptic drugs, and 4 children remain with uncontrolled seizures. All children have mental retardation and motor disabilities as well as symptoms of brainstem dysfunction. CONCLUSIONS: Our findings indicate that severe infantile thiamine deficiency may result in epilepsy.

The formation of calcium lactate crystals is responsible for concentrated acid whey thickening.

The use of spray drying for dehydration of acid whey is generally limited by the appearance of uncontrolled thickening and solidifying of the whey mass during the lactose crystallization step. The origin of this physical change is still unknown and probably linked to complex interactions between physical properties and chemical composition of these products. To understand this phenomenon, we simulated the thickening of concentrated acid whey on a laboratory scale by measuring the flow resistance changes as a function of time and whey composition. The thickening process was characterized by an amplitude of torque and a lag time (induction time). Thickening of lactic acid whey concentrate occurred regardless of the presence of whey proteins or lactose crystals. Moreover, this work clearly demonstrated that the thickening process was due to the formation of filamentous structures corresponding to calcium lactate crystals and showed a large dependence on calcium and lactate contents, pH, and phosphate concentration.

Familial Mediterranean fever: effects of genotype and ethnicity on inflammatory attacks and amyloidosis.

OBJECTIVE: The gene causing familial Mediterranean fever (FMF)-an autosomal recessive disease characterized by recurrent short episodes of fever associated most commonly with peritonitis, pleuritis, and arthritis-has recently been found and several mutations identified. The most severe complication of the disease is amyloidosis, which can lead to renal failure. The aim of this study was to investigate the role of genetic versus nongenetic factors on the phenotype as well as on the development of amyloidosis in FMF in a large and heterogeneous group of patients. METHODOLOGY: We studied 382 patients from 4 ethnic origins living in different environments: North African Jews, other Jews, Turks, Armenians living in the United States, and Armenians from Yerevan, Armenia. Information regarding amyloidosis was available for 371 patients. We examined the association between the mutation M694V and the development of amyloidosis, and we also compared the clinical characteristics of the inflammatory attacks in patients from different ethnic origins, while controlling for the type of mutation. RESULTS: A significant association was found between amyloidosis and the most common mutation in exon 10 of the FMF gene (MEFV), M694V (for M694V homozygotes, relative risk = 1.77; 95% CI = 1.16-2.71). Amyloidosis was present in 44 of 171 homozygous FMF patients (25.7%), in 22 of 143 compound heterozygous FMF patients (15.4%), and in 7 of 57 patients carrying other mutations (12.3%). In homozygotes for M694V who had not been treated with colchicine before 20 years of age, the risk of amyloidosis developing before this age was 61.0%. In our series, there were no cases of amyloidosis in 16 patients carrying the common mutation E148Q. We found that the type and severity of the FMF inflammatory symptoms were associated with both the genotype and the country of residence of the patient. CONCLUSIONS: In the light of the high frequency of amyloidosis in homozygotes for the mutation M694V, colchicine treatment should be given to this group irrespective of the severity of the inflammatory attacks to prevent the development of amyloidosis. Our findings also suggest that factors other than genotype, such as environment or genes other than MEFV, play a role in the determination of the severity of the inflammatory attacks in FMF. amyloidosis, specific mutation, phenotype-genotype correlation, ethnicity.

Phenotype-genotype correlation in familial Mediterranean fever: evidence for an association between Met694Val and amyloidosis.

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent attacks of inflammation of serosal membranes. Amyloidosis is the most severe complication of the disease. The aim of this study was to investigate the genotype-phenotype correlation and specifically the association between amyloidosis and the four common mutations in exon 10 of the gene causing FMF (MEFV) in a total of 83 FMF families from three ethnic groups: North African Jews, Armenians and Turks. A significant association was found between amyloidosis and the specific mutation at the MEFV gene: Met694Val (RR = 1.41, P = 0.02). Amyloidosis was present in 18 out of 87 homozygous FMF patients (20.7%) and in only two out of the 41 compound heterozygous FMF patients (4.9%). No patients carrying other mutations had amyloidosis. There was no significant association between the various mutations and the type or severity of the FMF symptoms. This finding underscores the importance of performing molecular studies on all suspect FMF patients. In addition to providing accurate diagnosis, these tests allow identification of presymptomatic genetically affected individuals, detection of carriers and assessment of the risk for amyloidosis in later life.

Effects of albumin on ionized calcium in vitro.

Human serum albumin is used to treat hypoproteinemia in neonates or used as a volume expander. We tested the hypothesis that the addition of human serum albumin to neonatal serum decreases the serum ionized calcium (iCa) concentration. Concentrated human serum albumin was added to 12 placental and cord serum samples to reach seven incremental concentrations from 0 to 20.0 g/L. Serum iCa concentration decreased significantly with the addition of serum albumin. From multiple regression analysis, the effect of albumin addition on serum iCa concentration had more marked effects at high baseline albumin or low baseline Ca concentrations. From this in vitro study, we speculate that fast infusion of albumin in human neonates has the potential for acutely lowering serum iCa concentration.

Fixed drug eruption following rifampin treatment.

A case of fixed drug eruption (FDE) associated with ingestion of rifampin in a young physician is reported. The drug was prophylactically administered because of a previous close contact with a meningococcemic patient. The eruption consisted of two solitary painless purplish lesions located over the extensor surface of the left forearm, characteristic of FDE, except for the absence of residual hyperpigmentation. It is suggested that slight lesions of FDE could be unnoticed or misdiagnosed, thus raising the possibility that such skin reactions to rifampin are more frequent than is reflected in the literature.