Doble tormenta tiroidea y arrítmica en una gestante con múltiples taquicardias refractarias mediadas por un haz de kent intermitente ablacionado exitosamente

La tormenta tiroidea es un estado crítico y poco frecuente que condiciona la disfunción de múltiples órganos por el efecto del exceso de las hormonas tiroideas, esta disfunción endócrina tiene una elevada mortalidad y genera manifestaciones típicas como la taquicardia, fiebre, alteraciones gastrointestinales, cardiovasculares y del sistema nervioso central. El embarazo se ha asociado con un incremento en la incidencia de arritmias. Necesitan un tratamiento inmediato con drogas antiarrítmicas, cardioversión eléctrica o cesárea de urgencia. El WPW es una anormalidad cardiaca congénita que consiste en la presencia de un haz anómalo (Haz de Kent) que evita el sistema normal de conducción uniendo directamente aurículas y ventrículos. Veremos el caso de una gestante de 32 semanas que presenta un cuadro de tormenta tiroidea y múltiples episodios de taquicardia paroxística supraventricular (TPS), de tórpida y sombría evolución clínica mediada por un haz anómalo de Kent intermitente. Es evidente que la tormenta tiroidea en el contexto de la gestación produjo cambios en las propiedades electrofisiológicas del haz anómalo de Kent intermitente lo cual propició el desarrollo de múltiples taquicardias paroxísticas supraventriculares refractarias a la cardioversión eléctrica y farmacológica. Tampoco mejoró con la tiroidectomía total, solamente cedió por completo con la ablación por catéter de radiofrecuencia del haz anómalo de Kent.

Thyroid storm is a critical and infrequent state that conditions the dysfunction of multiple organs due to the effect of excess thyroid hormones. This endocrine dysfunction has a high mortality and generates typical manifestations such as tachycardia, fever, gastrointestinal, cardiovascular and heart disorders, and the central nervous system. Pregnancy has been associated with an increased incidence of arrhythmias. They need immediate treatment with antiarrhythmic drugs, electrical cardioversion, or emergency caesarean section. WPW is a congenital cardiac abnormality that consists of the presence of an abnormal bundle (Kent bundle) that prevents the normal conduction system, directly joining the atria and ventricles. We will see the case of a 32-week pregnant woman who presented symptoms of thyroid storm and multiple episodes of paroxysmal supraventricular tachycardia (PST), with a torpid clinical course mediated by an abnormal intermittent Kent bundle. It is evident that the thyroid storm in the context of pregnancy produced changes in the electrophysiological properties of the intermittent Kent bundle, which led to the development of multiple PST refractory to electrical and pharmacological cardioversion. Moreover, it also did not improve with total thyroidectomy, only resolved completely with radiofrequency catheter ablation of the Kent bundle.

Effects of Rehabilitation Training Combined with Acupuncture on Upper Limb Function of Stroke Patients Based on Kinect / 医用生物力学

Objective To investigate the effect of rehabilitation training combined with acupuncture ( RTA) on upper limb function of stroke patients by Kinect. Methods Stroke patients with upper limb dysfunction werrandomly divided into control group (rehabilitation training) and treatment group ( RTA), with 15 cases in each group. The modified Barthel Index ( MBI), Fugl Meyer assessment ( FMA), and Wolf motor function test (WMFT) were compared between two groups before and after treatment. The changes in motor time ( MT), motor unit number (MUN), index of curvature (IC), elbow flexion angle (EFA), shoulder flexion angle (SFA),and shoulder adduction angle ( SAA) during three actions, namely, placing forearm on the table, extending elbow and drinking water, were evaluated by Kinect and then compared between two groups before and after treatment. Results After 6 weeks of intervention, the scores of MBI, FMA, WMFT and elbow extension in treatment group were higher than those in control group (P<0. 05). The scores of MBI, FMA, WMFT and three actions after treatment were higher than those before treatment (P<0. 05). For three actions, the improvement of MT, MUN, IC, EFA, SFA, and SAA in treatment group were better than those in control group ( P< 0. 05). Compared with pre-treatment, for the action of forearm placement on the table and elbow extension, both treatment group and control group showed an increase in EFA (P<0. 05), and a decrease in MT, MUN, IC, SFA and SA (P<0. 05). For the action of drinking water, both treatment group and control group showed an increase in EFA and SAA (P< 0. 05), and a decrease in MT, MUN, IC and SAA ( P< 0. 05). Conclusions RTA can improve the upper limb function of stroke patients. Kinect can accurately reflect the changes in upper limb function of stroke patients, and it is suitable for clinical work.

Family report of Wolf-Hirschhorn syndrome in twins / 中华神经科杂志

Objective:To explore the clinical manifestations and genetic characteristics of Wolf-Hirschhorn syndrome (WHS) to improve the ability of diagnosis and differential diagnosis of the disease.Methods:The clinical features and auxiliary examinations and treatment of a proband with WHS caused by microdeletion of 4p16.3 segment who admitted to the Third Affiliated Hospital of Zhengzhou University in December 2021 were recorded, and whole exome sequencing (WES) of the family was performed. The prognosis was followed up.Results:The female proband, 11 months old, presented with convulsions at the age of 8 months, with the characteristics of heat sensitivity and cluster seizures, and her identical twin sister had a similar medical history. Physical examination found malnutrition, retarded development, special face, prominent forehead, wide nasal bridge, small jaw, precordial murmur and grade 3/6 murmur in the whole period, hyperactivity of P2, and low limb muscle tone. The whole exon and copy number variation (CNV) test of the family revealed that the proband had a 1.99 Mb heterozygous deletion in the chromosome 4p16.3 segment, including WHSC1 (NSD2), WHSC2 (NEFLA) and other genes. Copy number variation sequencing (CNV-Seq) of the proband and her sister showed 1.97 and 1.92 Mb heterozygous deletion of chromosome 4p16.3, respectively. Genealogical analysis by quantitative polymerase chain reaction revealed that the CNV was de novo, and it was determined to be a pathogenic variant according to the American College of Medical Genetics and Genomics guidelines. The proband took sodium valproate orally, and her sister took oral sodium valproate, zonisamide, and levetiracetam successively, and at the same time they received family rehabilitation training. The age at the last follow-up was 1 year and 8 months. Neither of them had convulsions again in the past 3 months, but the developmental delay was obvious. Conclusion:WHS patients may present with growth retardation, epilepsy, Greek warrior helmet-like special face, and congenital heart disease, and may have microdeletions in the chromosome 4p16.3 segment.

Germination of fruits eaten by the maned wolf Chrysocyon brachyurus (Illiger, 1815) (Carnivora, Canidae) / Germinação de frutos consumidos pelo lobo-guará Chrysocyon brachyurus (Illiger, 1815) (Carnivora, Canidae)

Abstract Zoochory is a fundamental process that can be the main mechanism for seed and plant dispersal for many species. Mammals of the Carnivora order are among the most important dispersing agents; however, little is known regarding the role of canids as seed dispersers. Although the maned wolf (Chrysocyon brachyurus) has a potentially important role in seed dispersal, given its relatively high consumption of fruits, few studies have investigated the germination rate of ingested seeds. Here, we used seeds removed from the feces of two captive specimens (maned wolf treatment) and those directly collected from unconsumed fruits (control) in germination essays to evaluate the germination rate and emergence velocity index (IVE). We used mature fruits from five species of trees in the Cerrado and Atlantic Forest in a 5 (species) × 2 (method of seed collection) factorial arrangement. The passage of seeds through the digestive tract of the maned wolf favored the germination of Genipa americana and Psidium guajava, delayed germination of Psidium cattleianum, and maintained the germination of Plinia cauliflora and Ficus obtusifolia. Our results revealed that germination occurred for all tested plant species consumed by the maned wolf; therefore, this canid species has high dispersal potential and can be an important ally in the restoration of the Cerrado and Atlantic Forest-Cerrado contact zone ecosystems.

Resumo A zoocoria é um processo fundamental para muitas espécies vegetais, podendo ser o principal mecanismo de dispersão de sementes e plantas. Os mamíferos da ordem Carnivora estão entre os agentes dispersores mais importantes, contudo, pouca atenção tem sido dada ao papel dos canídeos como dispersores de sementes. Embora o lobo-guará (Chrysocyon brachyurus) tenha um papel potencialmente importante na dispersão de sementes dado o seu consumo relativamente elevado de frutos, poucos estudos investigaram a taxa de germinação de sementes ingeridas. Aqui utilizamos sementes retiradas de fezes de dois exemplares em cativeiro (tratamento do lobo-guará) e diretamente retiradas de frutos não consumidos (controle) em ensaios de germinação para avaliar a taxa de germinação e o índice de velocidade de emergência (IVE). Utilizamos frutos maduros de cinco espécies de árvores que ocorrem no Cerrado e Mata Atlântica formando um arranjo fatorial de 5 (espécie) × 2 (forma de coleta de sementes). A passagem das sementes pelo trato digestivo do lobo-guará favoreceu a germinação de duas espécies, Genipa americana e Psidium guajava, atrasou a germinação de uma espécie, Psidium cattleianum e não afetou a germinação de duas espécies, Plinia cauliflora e Ficus obtusifolia. Com base nos nossos resultados, particularmente a descoberta de que todas as espécies vegetais testadas consumidas pelo lobo-guará germinaram, concluímos que esta espécie de canídeo tem um elevado potencial de dispersão, sendo um importante aliado na restauração de ecossistemas do Cerrado e da zona de contato entre Cerrado e Mata Atlântica.

Wolff-Parkinson-White Apresentado como QRS Alternante e Outros Diagnósticos Diferenciais em uma Grande Coorte de Triagem de ECG Pré-Participação / Wolff-Parkinson-White Presenting as QRS Alternans and Other Differential Diagnoses in a Large Pre-Participation ECG Screening Cohort

Resumo Fundamento A síndrome de Wolff-Parkinson-White (WPW) é uma condição pró-arrítmica que pode exigir restrição de atividades extenuantes e é caracterizada por sinais de ECG, incluindo ondas delta. Observamos casos de padrões intermitentes de WPW apresentando-se como QRS alternante ('WPW alternante') em uma grande coorte de triagem de ECG pré-participação de homens jovens que se candidataram ao recrutamento militar. Objetivos Nosso objetivo foi determinar o padrão de WPW alternante, as características do caso e a prevalência de outros diagnósticos diferenciais relevantes apresentando-se como alternância de QRS em um ambiente de pré-participação. Métodos Cento e vinte e cinco mil cento e cinquenta e oito recrutas militares do sexo masculino prospectivos foram revisados de janeiro de 2016 a dezembro de 2019. Uma revisão de prontuários médicos eletrônicos identificou casos de WPW alternante e padrões ou síndrome de WPW. A revisão de prontuários médicos eletrônicos identificou casos de diagnósticos diferenciais relevantes que podem causar alternância de QRS. Resultados Quatro indivíduos (2,2%) apresentaram WPW alternante em 184 indivíduos com diagnóstico final de padrão ou síndrome de WPW. Dois desses indivíduos manifestaram sintomas ou achados eletrocardiográficos compatíveis com taquicardia supraventricular. A prevalência geral de WPW alternante foi de 0,003%, e a prevalência de WPW foi de 0,147%. As WPW alternantes representaram 8,7% dos indivíduos com QRS alternantes, e QRS alternantes tiveram prevalência de 0,037% em toda a população. Conclusões A WPW alternante é uma variante da WPW intermitente, que compreendeu 2,2% dos casos de WPW em nossa coorte de triagem pré-participação. Não indica necessariamente um baixo risco de taquicardia supraventricular. Deve ser reconhecido na triagem de ECG e distinguido de outras patologias que também apresentam QRS alternantes.

Abstract Background Wolff-Parkinson-White (WPW) syndrome is a proarrhythmic condition that may require restriction from strenuous activities and is characterized by ECG signs, including delta waves. We observed cases of intermittent WPW patterns presenting as QRS alternans ('WPW alternans') in a large pre-participation ECG screening cohort of young men reporting for military conscription. Objectives We aimed to determine the WPW alternans pattern, case characteristics, and the prevalence of other relevant differential diagnoses presenting as QRS alternans in a pre-participation setting. Methods One hundred twenty-five thousand one hundred fifty-eight prospective male military recruits were reviewed from January 2016 to December 2019. A review of electronic medical records identified cases of WPW alternans and WPW patterns or syndrome. Reviewing electronic medical records identified cases of relevant differential diagnoses that might cause QRS alternans. Results Four individuals (2.2%) had WPW alternans out of 184 individuals with a final diagnosis of WPW pattern or syndrome. Two of these individuals manifested symptoms or ECG findings consistent with supraventricular tachycardia. The overall prevalence of WPW alternans was 0.003%, and the prevalence of WPW was 0.147%. WPW alternans represented 8.7% of individuals presenting with QRS alternans, and QRS alternans had a prevalence of 0.037% in the entire population. Conclusions WPW alternans is a variant of intermittent WPW, which comprised 2.2% of WPW cases in our pre-participation screening cohort. It does not necessarily indicate a low risk for supraventricular tachycardia. It must be recognized at ECG screening and distinguished from other pathologies that also present with QRS alternans.

A Case of Wolf-Hirschhorn Syndrome with Early Rehabilitation Intervention from the Neonatal Period / The Japanese Journal of Rehabilitation Medicine

Wolf-Hirschhorn syndrome is a chromosomal aberration caused by a deletion of the distal short arm of chromosome 4, characterized by distinct craniofacial features, failure to thrive, psychomotor developmental retardation, epilepsy, and feeding disorders. We report a case of patient with Wolf-Hirschhorn syndrome who underwent interventional rehabilitation commencing from the neonatal period in the neonatal intensive care unit. The patient was born at gestational age of 38 weeks 0 days, weighing 1583 g, with an Apgar score of 4/9, and was diagnosed with partial monosomy of the short arm of chromosome 4. Postnatal inspiratory stridor exacerbation was noted for which high-flow nasal cannula therapy was initiated. Rehabilitation commenced on the 18th day after the infant's birth, to promote sensorimotor development. Initially, the trunk was in a low muscle tension and unstable state. Therefore, we first prescribed rest followed by sensorimotor rehabilitation. When the infant's clinical condition stabilized, we performed prone and anti-gravity hugging exercises to improve the low trunk tension. Breastfeeding evaluation began 56 days after birth, when the respiratory condition improved. We practiced feeding the infant orally, in collaboration with doctors and nurses, to reduce bending and stabilize the posture when raising the mandible. The infant was gradually able to feed orally and gained weight. Thereafter, he was discharged 141 days after birth. This report concluded that rehabilitation intervention from the neonatal period, in collaboration with the multidisciplinary team and patient's family, contributed to initiation of oral feeding, improvement of sensorimotor development, and smooth transition to home care.

Research progress on chemical composition and pharmacological effects of Poria cocos and predictive analysis on quality markers / 中草药

Poria cocos is a traditional Chinese medicine with homology of medicine and food in China. It has the effects of promoting diuresis, eliminating dampness, invigorating spleen, calming heart and so on. It is widely used in medicine, food and health care products. With the in-depth study of P. cocos, its triterpenes, polysaccharides and other major chemical components, as well as its wide range of pharmacological effects and application development research have attracted much attention. This paper systematically reviewed the chemical components and pharmacological effects of P. cocos, according to the concept of quality markers, the quality markers of P. cocos were predicted and analyzed from the aspects of the biosynthetic approach and specificity of chemical components, traditional medicinal efficacy, traditional medicinal properties, measurable components, different processing methods and so on, which provides a scientific basis for quality evaluation and product development of P. cocos.

Prescription regularity of traditional Chinese medicine for treating alcoholic liver disease based on traditional Chinese medicine inheritance support system / 中草药

Objective: To summarize the prescription rules of traditional Chinese medicine (TCM) for treating alcoholic liver disease (ALD) based on TCM inheritance support system (V2.50). Methods: The literatures about TCM prescriptions for treating ALD were collected from CNKI, Wanfang database, and VIP database. The TCM inheritance platform system was used to analyze the prescription rules of TCM in the treatment of alcoholic liver disease. Results: Statistics showed that the majority of prescriptions were used to treat alcoholic fatty liver, alcoholic hepatitis, and alcoholic cirrhosis. Through "frequency statistics" analysis, 107 prescriptions were found involving 149 flavors of TCM, with a cumulative frequency of 1 195 times. Twenty-three Chinese medicines with a frequency of ≥ 15 times were used, and the cumulative frequency was 737 times (62%). The most frequently used medicines were blood-activating and stasis-removing drugs, water-diffusing and damp-permeating drugs, tonics, heat-clearing drugs, antialcoholic poisons and qi-regulating drugs. The commonly used doses of Salvia miltiorrhiza, Poria cocos, Bupleurum chinense, Glycyrrhiza uralensis, Atractylodes macrocephala, Alismatis Rhizoma, and Curcumae Radix in the top 10 medicines ranked in the frequency of medication accorded with the prescribed doses in the Pharmacopoeia of the People's Republic of China (2015 edition), while Crataegi Fructus, Artemisiae Scopariae Herba, and Puerariae Lobatae Radix exceeded the prescribed doses. In the frequency analysis of drug pairs, the combination of S.miltiorrhiza and B. chinense was the most widely used. According to the association rules of drug combination, the correlation between Curcumae Radix and S. miltiorrhiza was the strongest, that was, the probability of S. miltiorrhiza appearing with the emergence of Curcumae Radix was 88%. From the network display chart, it was indicated that S. miltiorrhiza and P. cocos were the main herbs for treatment. Through unsupervised entropy hierarchical clustering algorithm, 14 core combinations for new clustering were extracted, and seven new prescriptions can be obtained by further clustering. Conclusion: The basic principles of TCM treatment of ALD include promoting blood circulation and removing blood stasis, removing dampness, tonifying, detoxifying alcohol, and promoting qi, and with "protecting spleen and stomach function" as its purpose, which accords with the theoretical basis of traditional Chinese medicine in treating alcoholic liver disease. Core combinations and new prescriptions provide references for clinical drug use and new drug research and development, but new prescriptions must be further evaluated with the combination of traditional Chinese medicine theory and clinical practice.

Anatomia óssea e muscular do antebraço e mão de Chrysocyon brachyurus (carnivora, canidae) / Bone and muscular anatomy of the antibone and hand of Chrysocyon brachyurus (carnivora, canidae)

O lobo-guará Chrysocyon brachyurus Illiger, 1815, é o maior canídeo da América do Sul, pesa cerca de 25 kg quando adulto e está ameaçado de extinção. Descrições anatômicas contribuem para a complementação das informações sobre espécies silvestres e para implicações conservacionistas, clínicas e cirúrgicas. Objetivou-se descrever os ossos e os músculos do antebraço e mão do lobo-guará. A preparação das peças foi feita a partir dos métodos usuais de dissecação, em animais preservados em solução de formol a 10%. Os espécimes pertencem ao acervo didático do Laboratório de Ensino e Pesquisa em Animais Silvestres da UFU e são provenientes de indivíduos atropelados. Os ossos descritos foram: rádio, ulna, ossos cárpico acessório, cárpico ulnar e cárpico intermédio; ossos cárpicos I, II, III e IV; ossos metacárpicos I, II, III, IV, V; falanges proximais, falanges médias e falanges distais do primeiro ao quinto dedo. Os músculos observados foram: extensor radial do carpo; pronador redondo; braquiorradial; extensor comum dos dedos; extensor ulnar do carpo; extensor lateral dos dedos;supinador; abdutor longo do dedo I; flexor radial do carpo; flexor profundo dos dedos; flexor superficial dos dedos; flexor ulnar do carpo; pronador quadrado; interflexor; lumbricais; abdutor curto dos dedos I e II e flexor curto do dedo I.

The maned wolf Chrysocyon brachyurus (Illiger, 1815) is the largest canid in South America, weighs about 25 kg as an adult and is threatened of extinction. Anatomical descriptions contribute to the complementation of information on wild species and for conservation, clinical and surgical implications. The purpose of this study was to describe the bones and muscles of the forearm and hand of the maned wolf. The methodology was through the usual dissecting methods in animals preserved in 10% formalin solution. The animals belong to the didactic collection of the Laboratory of Teaching and Research in Wild Animals of the UFU and come from run over. The bones evaluated were: radius, ulna, carpal accessory, carpi ulnar and carpi intermedium; carpal bones I, II, III and IV; metacarpal bones I, II, III, IV, V; proximal phalanges, middle phalanges and distal phalanges from first to fifth finger. The muscles observed were: radial extensor carpal; pronator round; brachioradial; common extensor of fingers; ulnar carpal extensor; lateral extensor of the fingers; supinator; abductor long finger I; flexor carpi radialis; flexor deep fingers; superficial flexor of the fingers; ulnar flexor of the carpus; square pronator; interflexor; lumbrils; short abductor of fingers I and II and short flexor of finger I.

Anatomia óssea e muscular do antebraço e mão de Chrysocyon brachyurus (Carnivora, Canidae) / Bone and muscular anatomy of the antibone and handof Chrysocyon brachyurus (Carnivora, Canidae)

O lobo-guará Chrysocyon brachyurus Illiger, 1815, é o maior canídeo da América do Sul, pesa cerca de 25 kg quando adulto e está ameaçado de extinção. Descrições anatômicas contribuem para a complementação das informações sobre espécies silvestres e para implicações conservacionistas, clínicas e cirúrgicas. Objetivou-se descrever os ossos e os músculos do antebraço e mão do lobo-guará. A preparação das peças foi feita a partir dos métodos usuais de dissecação, em animais preservados em solução de formol a 10%. Os espécimes pertencem ao acervo didático do Laboratório de Ensino e Pesquisa em Animais Silvestres da UFU e são provenientes de indivíduos atropelados. Os ossos descritos foram: rádio, ulna, ossos cárpico acessório, cárpico ulnar e cárpico intermédio; ossos cárpicos I, II, III e IV; ossos metacárpicos I, II, III, IV, V; falanges proximais, falanges médias e falanges distais do primeiro ao quinto dedo. Os músculos observados foram: extensor radial do carpo; pronador redondo; braquiorradial; extensor comum dos dedos; extensor ulnar do carpo; extensor lateral dos dedos; supinador; abdutor longo do dedo I; flexor radial do carpo; flexor profundo dos dedos; flexor superficial dos dedos; flexor ulnar do carpo; pronador quadrado; interflexor; lumbricais; abdutor curto dos dedos I e II e flexor curto do dedo I.

The maned wolf Chrysocyon brachyurus (Illiger, 1815) is the largest canid in South America, weighs about 25 kg as an adult and is threatened of extinction. Anatomical descriptions contribute to the complementation of information on wild species and for conservation, clinical and surgical implications. The purpose of this study was to describe the bones and muscles of the forearm and hand of the maned wolf. The methodology was through the usual dissecting methods in animals preserved in 10% formalin solution. The animals belong to the didactic collection of the Laboratory of Teaching and Research in Wild Animals of the UFU and come from run over. The bones evaluated were: radius, ulna, carpal accessory, carpi ulnar and carpi intermedium; carpal bones I, II, III and IV; metacarpal bones I, II, III, IV, V; proximal phalanges, middle phalanges and distal phalanges from first to fifth finger. The muscles observed were: radial extensor carpal; pronator round; brachioradial; common extensor of fingers; ulnar carpal extensor; lateral extensor of the fingers; supinator; abductor long finger I; flexor carpi radialis; flexor deep fingers; superficial flexor of the fingers; ulnar flexor of the carpus; square pronator; interflexor; lumbrils; short abductor of fingers I and II and short flexor of finger I.

Síndrome de Wolf-Hirschhorn: Descripción de cinco casos caracterizados por microarrays de polimorfismos de nucleótido único / Wolf-Hirschhorn syndrome: Description of five cases characterized by means of single nucleotide polymorphism microarrays

El síndrome de Wolf-Hirschhorn es una entidad polimalformativa debida a la microdeleción en la región distal del brazo corto del cromosoma 4 (4p16.3), el cual produce una serie de manifestaciones clínicas, que pueden variar dependiendo del tipo y tamaño del defecto genético en este síndrome de genes contiguos. Se presentan cinco pacientes, tres de ellos de sexo femenino, todos con los hallazgos clínicos primordiales, con rasgo facial característico de "apariencia en casco de guerrero griego", retraso en el crecimiento y del desarrollo psicomotor. Además de la deleción parcial en la región distal del brazo corto del cromosoma 4, en dos pacientes, se encontraron alteraciones genéticas adicionales, mediante el uso de microarrays de polimorfismos de nucleótido único. Se resaltan las características clínicas del síndrome de Wolf-Hirschhorn con la finalidad de orientar el diagnóstico, brindar una atención médica interdisciplinaria y, a través de su confirmación, brindar un adecuado asesoramiento genético familiar.

Wolf-Hirschhorn syndrome is a polymalformative entity due to the microdeletion in the distal region of the short arm of chromosome 4 (4p16.3), which produces a series of clinical manifestations that can vary depending on the type and size of the genetic defect in this contiguous gene syndrome. Five patients are presented, three of them female, all with the primary clinical findings, characterized by "Greek warrior helmet appearance" facial feature, growth retardation and psychomotor development delay. In addition to the partial deletion in the distal region of the short arm of chromosome 4, two additional genetic alterations were found in two patients, through the use of single nucleotide polymorphism arrays. The clinical characteristics of Wolf-Hirschhorn syndrome are highlighted in order to guide the diagnosis, provide interdisciplinary medical care and, through its confirmation, provide adequate family genetic counseling.

Granuloma anular generalizado y fenómeno isotópico de Wolf / Generalized annular granuloma and Wolf''s isotopic response

El granuloma anular (GA) es una dermatosis inflamatoria granulomatosa, generalmente asintomática, con distintas formas de presentación clínica, que puede distribuirse de forma localizada o generalizada. su etiología es incierta pero se la ha vinculado a factores desencadenantes como traumatismos, fármacos, diabetes mellitus, tiroideopatías, neoplasias, infecciones virales (herpes simple y varicela zóster) y excepcionalmente puede aparecer en la misma localización donde previamente aconteció otra enfermedad con la cual no guarda relación alguna (fenómeno isotópico de Wolf). En su patogenia intervendrían mecanismos de hipersensibilidad retardada y de estrés oxidativo celular. Existen múltiples terapias con respuesta variable y se ha observado autorresolución en casi el 75% de los casos. Presentamos el caso de una mujer de 64 años de edad, hipotiroidea de larga data, con un granuloma anular generalizado que apareció sobre cicatrices residuales de un herpes zóster (fenómeno isotópico de Wolf) y su respuesta al tratamiento con Vitamina E por vía oral

Granuloma annulare (GA) is a granulomatous inflammatory dermatosis, usually asymptomatic, with different forms of clinical presentation, which can be localized or generalized. Its etiology is uncertain but has been linked to triggers such as trauma, drugs, diabetes mellitus, thyroid disease, neoplasms and viral infections (herpes simplex, varicella-zoster). Exceptionally, it may appear in the same location where another disease has previously occurred but with no relationship to it (Wolf isotopic response). In their pathogenesis, delayed hypersensitivity mechanisms and cellular oxidative stress would intervene. There are multiple therapies with variable response, and autorresolution has been observed in almost 75% of the cases. We present the case of a 64-year-old woman with a long history of hypothyroidism, with diagnosis of generalized granuloma annulare on residual scars of Herpes Zoster (Wolf 's isotopic response) and its response to oral Vitamin E.

Prenatal diagnosis of Wolf-Hirschhorn syndrome: a case report / 中华围产医学杂志

This study reported a case of fetal developmental retardation indicated by ultrasound from 17+2 to 34+5 gestations.Single nucleotide polymorphism (SNP) array was performed to detect the copy number variation in the whole genome for the fetus and parents.A 2.42 Mb deletion at 4p16.3 was found in the fetus,but in neither parents,which suggesting a de novo mutation.Thus,the fetus was finally diagnosed with Wolf-Hirschhorn syndrome.No obvious'Greek warrior helmet'appearance or other facial deformity was observed in the delivered fetus.

Monosaccharide analysis and fingerprinting identification of polysaccharides from Poria cocos and Polyporus umbellatus by HPLC combined with chemometrics methods / 中草药·英文版

Objective: Poria cocos and Polyporus umbellatus are similar medicinal fungi in traditional Chinese medicines. A method for fingerprint analysis of monosaccharide composition of polysaccharides by HPLC combined with chemometrics methods has been developed for characterization and discrimination of them in this research. Methods: The polysaccharides were extracted by decocting in water, and then completely hydrolyzed with hydrochloride. Monosaccharides in the hydrolyzates were derivatized with 1-phenyl-3-methyl-5-pyrazolone (PMP) for HPLC analysis. More than 20 batches of P. cocos and P. umbellatus from different regions were analyzed. Results: The fingerprints of P. cocos showed five common characteristic peaks, which were identified by comparing with the reference substances. The five peaks corresponded to the derivatives of mannose, ribose, glucose, galactose, and fucose. At the same time, the fingerprints of P. umbellatus showed eight common characteristic peaks, of which seven were identified as the derivatives of mannose, ribose, rhamnose, glucose, galactose, xylose, and fucose. Moreover, the similarity of their fingerprints was respectively calculated by the Similarity Evaluation System for Chromatographic Fingerprint of TCM published by China Pharmacopoeia Committee (Version 2004A). And the data were further processed by hierarchical cluster analysis (HCA) and principal component analysis (PCA). The similarity evaluation and HCA indicated that there were no significant difference in P. cocos or P. umbellatus samples from different geographical regions, but PCA was performed to characterize the difference in monosaccharide constituents between P. cocos and P. umbellatus, and linear discriminant analysis (LDA) showed the overall correct classification rate was 100%. Conclusion: The fingerprint analysis method of monosaccharide composition of water-soluble polysaccharides can distinguish P. cocos and P. umbellatus, and can be applied for the authentication or quality control for P. cocos and P. umbellatus.

Non-polysaccharide chemical constituents of Poria cocos and their anti-complementary activity / 中草药

Objective: To investigate the non-polysaccharide chemical constituents of Poria cocos and their anti-complementary activity. Methods: The anti-complementary bioassay-guided isolation was carried out with the hemolysis test as guide. All isolates were evaluated for their in vitro anti-complementary activities on the classical pathway. The structures were identified by various spectroscopic data including ESI-MS, 1H-NMR, and 13C-NMR data. Results: Eleven compounds were isolated from the EtOAc fraction of P. cocos extracts, including stigmasterol (1), lupeol (2), oleanolic acid (3), ursolic acid (4), polyporenic acid C (5), tumulosic acid (6), dehydrotumulosic acid (7), 3-epi-dehydrotumulosic acid (8), pachymic acid (9), dehydropachymic acid (10), and dehydrotrametenolic acid (11). Compounds 1-4 were obtained from this plant for the first time, and compounds 3-11 showed the anti-complementary activity in different degrees. Conclusion: Triterpenoid acids are the main anti-complementary constituents in the chemical constituents of P. cocos non-polysaccharides (CH50 0.10-0.27 g/L).

Discovery of quality markers and quality evaluation of Poria cocos based on “in vitro-in vivo” multidimensional chemical group associated network / 中草药

Objective: The quality markers of Poria cocos were identified based on the “in vitro-in vivo” multidimensional chemical group associated network and in vivo pharmacokinetics, and the mass spectrometry method for quantitative detection of quality markers was established to evaluate the quality of P. cocos. Methods: A quantitative analysis method of triterpenoids in P. cocos was established by ultra-high performance liquid chromatography-triple quadruple tandem mass spectrometry (UHPLC-MS/MS) while dioscin was chosen as internal reference substance. The pharmacokinetic curves of active ingredients in vivo were drawn. Seven quality markers of P. cocos including dehydrotumulosic acid, tumulosic acid, and poricoic acid B were identified based on the results of pharmacokinetics. The content of high content components and quality markers in 10 batches of P. cocos were detected and used as variables for cluster analysis of 10 batches of P. cocos. Results: There were differences in the results of clustering analysis with different indexes as variables in evaluating the quality of P. cocos. Conclusion: To a certain extent, the method of quality control of P. cocos with high content components is one-sided. In order to comprehensively and accurately control the quality of P. cocos, it is necessary to take into full account the in vivo and in vitro changes and the in vivo dynamic process of P. cocos composition, and select the effective quality markers related to its pharmacodynamics for its quality control.

Actividad solar y hospitalizaciones por episodios depresivos en Chile / Solar Activity and hospitalizations for depressive episodes in Chile

Resumen Introducción: Este trabajo presenta las relaciones entre la actividad solar y la incidencia de hospitalizaciones por cuadros depresivos en el registro del Ministerio de Salud de Chile donde se registran todos los egresos hospitalarios entre los años 2001 a 2008. La actividad solar está representada por el número de Wolf, mediante la fórmula R = K (10g+f), donde g corresponde al grupo de manchas solares y f es el número total de manchas solares. Método: Las bases de datos del Ministerio de Salud muestran todos los egresos hospitalarios, tanto de causa psiquiátrica como por otros diagnósticos, incluyendo tantos hospitales públicos como también privados. Para el análisis muestral se seleccionaron los casos que tuvieron como diagnóstico algún episodio depresivo según la clasificación CIE 10 desde F320 hasta F339, ambos incluidos. Se examinó la correlación entre la incidencia anual de hospitalizaciones por depresiones y el número de Wolf promedio para ese determinado año durante el período comprendido entre los años 2001 a 2008, ambos inclusive, lo que corresponde aproximadamente a la mitad de un ciclo solar de 16 años. Resultados: Una menor intensidad de la actividad solar se correlaciona con una mayor frecuencia de hospitalizaciones por depresión en Chile. Conclusiones: Los desórdenes depresivos poseen una correlación inversa significativa con la actividad solar.

Introduction: This paper presents the relations between solar activity and the incidence of hospitalizations for depressive disorder found in the register of the Ministry of Health of Chile, where all the hospital discharges from 2001 to 2008 are kept. Solar activity is represented by the Wolf number, with the formula R = K (10g+f), where g refers to the group of sunspots and f is the total number of sunspots. Method: The databases of the Ministry of Health show all the hospital discharges, whether for psychiatric causes or other diagnoses, including data from public as well as private hospitals. For the sample analysis, two cases diagnosed as depressive disorder according to the classification CIE 10, ranging from F320 to F339, both included, were selected. The correlation between annual incidence of hospitalizations for depression and the average of the Wolf number for that particular year between the period spanning from 2001 to 2008, both included, which corresponds to approximately half of a solar cycle of 16 years. Results: Less intensity of solar activity correlates to a higher frequency of hospitalizations for depression in Chile. Conclusions: Depressive disorders have a significant inverse correlation to solar activity.

Simultaneous quantitative analysis of four triterpenes in Poria Cocos from different habitats by QAMS / 中草药

Objective To establish a new method and validate its feasibilities by the simultaneous quantitative assay of four triterpenes in Poria cocos. Methods A new quality evaluation method of multi-components by single marker (QAMS) was established and validated for P. cocos. Pachymic acid (PA), dehydropachymic acid (DPA), dehydrotumulosic acid (DTA), and dehydrotrametenolic acid (DMA) were selected as analytes while pachymic acid was chosen as internal reference substance to evaluate the quality. The relative correction factor (RCF) of pachymic acid to the other three triterpenes were calculated. The method was evaluated by the comparison of quantity between external standard method and QAMS method. Results The contents of four triterpenes in 17 batches of P. cocos from QAMS method were not significantly different from those from external standard method. Conclusion The method with a single marker is accurate and feasible to determine PA, DPA, DTA, and DMA when some authentic standard substance are unavailable.

Malignant pancreatic gastrinoma in a maned wolf (Chrysocyon brachyurus): case report / Gastrinoma pancreático maligno em lobo-guará (Chrysocyon brachyurus): relato de caso

A captive 12-year-old female maned wolf (Chrysocyon brachyurus), previously diagnosed with visceral leishmaniasis, developed severe myiasis in the right ear, followed by prostration and death. A firm solid nodule, measuring 1 x 3cm, was grossly observed in the pancreas. Histologically, there was neoplastic proliferation in the pancreas and pancreatic lymph node. Neoplastic cells had a neuroendocrine pattern, and were positive for gastrin and pancreatic polypeptide by immunohistochemistry. The wolf had also ulcerative gastritis. To the best of our knowledge is the first reported case gastrinoma in this species.(AU)

Uma fêmea lobo-guará de cativeiro (Chrysocyon brachyurus), diagnosticada previamente com leishmaniose visceral, desenvolveu miíase grave na orelha direita, seguida por prostação e morte. Um nódulo sólido, firme e medindo 1 x 3cm, foi macroscopicamente observado no pâncreas. Histologicamente, observou-se proliferação neoplásica no pâncreas e linfonodo pancreático. As células neoplásicas tinham um padrão neuroendócrino e foram positivas para gastrina e polipeptideo pancreático por imuno-histoquímica. A loba tinha também gastrite ulcerativa. Este é o primeiro caso descrito de gastrinoma nessa espécie.(AU)

Effect of Acupuncture plus Rehabilitation Training on Upper Limb Functional Recovery in Stroke Patients / 上海针灸杂志

Objective To investigate the effect of acupuncture plus rehabilitation training on upper limb functional recovery in convalescent stroke patients.Methods One hundred and three convalescent stroke patients with upper limb motor dysfunction were randomized to a treatment group (54 cases) and a control groups (49 cases). Both group received conventional rehabilitation training. In addition, the treatment group received along-meridian syndrome differentiation-based acupuncture and the control group, sham acupuncture. Upper limb Fugl-Meyer Assessment (U-FMA) score, the Wolf Motor Function Test (WMFT) score, the modified Barthel Index (MBI) score and the 36-Item Short Form Health Survey (SF-36) score were recorded in the two groups of patients before and at the end of treatment and at the 3-month follow-up.Results In the two groups there were statistically significant differences in the U-FMA score, the WMFT score and the MBI score at the end of treatment and the 3-month follow-up compared with before (P>0.05 orP<0.01). At the end of treatment and the 3-month follow-up there were statistically significant differences in the U-FMA score, the WMFT score and the MBI score between the treatment and control groups (P<0.05 orP<0.01). The scores of SF-36 items except social functioning and bodily pain had statistically significant differences within the two groups at the end of treatment and the 3-month follow-up compared with before and between the two groups at the end of treatment and the 3-month follow-up (P<0.01 orP<0.05).Conclusion Acupuncture plus rehabilitation training can markedly improve upper limb function in stroke patients.