Síndrome de Rapunzel: atadura sicológica y simulación orgánica / Rapunzel syndrome: psychological bondage and organic simulation

Introducción. El síndrome de Rapunzel es una entidad infrecuente, que se presenta como un tricobezoar a causa de una aglomeración de cabello acumulado dentro del tracto gastrointestinal, por lo que simula otras patologías quirúrgicas. Caso clínico. Paciente femenina de 10 años de edad, con tricotilomanía y tricofagia, dolor abdominal y síntomas inespecíficos de obstrucción intestinal de ocho meses de evolución. Al examen físico se encontró abdomen con distensión y masa palpable en epigastrio y mesogastrio. La ecografía permitió hacer el diagnóstico de tricobezoar gástrico extendido hasta el intestino delgado, por lo que se llevó a cirugía para gastrotomía y se extrajo el tricobezoar, con evolución satisfactoria de la paciente. El abordaje integral permitió conocer la atadura sicológica por posible maltrato infantil. Resultado. La paciente tuvo una evolución satisfactoria y se dio egreso al quinto día de hospitalización. Actualmente se encuentra en seguimiento por sicología, siquiatría infantil y pediatría. Discusión. El caso clínico denota la importancia en reconocer situaciones de presentación infrecuente en pediatría, que puedan estar asociadas a alteraciones sicológicas o presunción de maltrato infantil y que se presenten como una condición orgánica recurrente que simule otras patologías abdominales frecuentes en la infancia. El retraso diagnóstico puede conducir a un desenlace no deseado con complicaciones. Conclusión. Se hace mandatorio el manejo integral del paciente pediátrico y aumentar la sensibilidad para reconocer situaciones de presunción de maltrato infantil, sobre todo en pacientes con una condición orgánica quirúrgica recurrente.

Introduction. Rapunzel syndrome is an uncommon condition that manifests as trichobezoars, which are hair bundles in the stomach or small intestine that can mimics other surgical illnesses. Multiple complications can arise from delayed diagnosis and treatment. Clinical case. A 10-year-old female patient with trichotillomania and trichophagia, with abdominal pain and nonspecific symptoms of intestinal obstruction of eight months of evolution. Physical examination revealed epigastric tenderness and a solid mass was palpable in the mesogastric and epigastric region. An abdominal ultrasound showed gastric trichobezoar that extended into the small intestine. A gastrotomy was performed and the trichobezoar was extracted with satisfactory evolution of the patient. The comprehensive approach allowed knowing the psychological bond due to possible child abuse. Results. The patient had a satisfactory evolution and was discharged on the fifth day of hospitalization. He is currently being monitored by psychology, child psychiatry and pediatrics. Discussion. This clinical case highlights the importance of recognizing situations that seldom present in pediatrics, which may have a psychological aspect due to the presumption of child abuse, and which present as a recurrent organic condition simulating other frequent abdominal pathologies in childhood; all of which may lead to an unwanted outcome due to diagnostic delay. Conclusion. The comprehensive management of the pediatric patient is mandatory to recognize situations of presumed child abuse, in the face of a recurrent surgical conditions.

Síndrome de Rapunzel asociado a perforación intestinal. Caso clínico / Rapunzel syndrome associated with intestinal perforation. Clinical case

Introducción. Los tricobezoares ocurren de forma frecuente en niñas y adolescentes, y se asocian a trastornos psicológicos como depresión, tricotilomanía o tricofagia. Caso clínico. Se presenta una paciente adolescente con síndrome de Rapunzel, con hallazgo adicional de perforación yeyunal debido al tricobezoar. Discusión. Dentro de las complicaciones de los tricobezoares se reporta invaginación intestinal (principalmente de yeyuno), apendicitis, obstrucción biliar, neumonía, pancreatitis secundaria y perforación, esta última como ocurrió en nuestra paciente. Conclusión. En pacientes mujeres adolescentes con dolor abdominal o abdomen agudo, se debe tener en cuenta el diagnóstico de síndrome de Rapunzel, así como sus probables complicaciones

Introduction. Trichobezoars occur frequently in young and adolescent girls, and are associated with psychological disorders such as depression, trichotillomania, or trichophagia. Clinical case. An adolescent patient with Rapunzel syndrome is presented, with an additional finding of jejunal perforation due to the trichobezoar. Discussion. Among the complications of trichobezoars, intussusception is reported (mainly of the jejunum), appendicitis, biliary obstruction, pneumonia, secondary pancreatitis, and perforation, the latter as occurred in our patient. Conclusion. In adolescent female patients with abdominal pain or acute abdomen, the diagnosis of Rapunzel syndrome should be taken into account, as well as its probable complications

Tricobezoar gástrico gigante: reporte de un caso clínico-patológico

Un tricobezoar es una masa compacta de pelo que se aloja generalmente en el estómago. Es frecuente en mujeres jóvenes que comen su propio pelo (tricofagia) debido a trastornos psicológicos. Presentamos el caso de una adolescente de 13 años de edad quien es traída por emergencia por presentar dolor abdominal persistente desde hace 3 días. La ecografía abdominal fue sugestiva de tricobezoar, hallazgo que fue confirmado mediante una endoscopia digestiva alta. Desde hace dos años antes de su ingreso, presentaba, masticación de cabello y consumo del mismo que asocia a viaje de su hermano al extranjero y a la separación de sus padres. Al noveno día, se realizó laparotomía exploratoria con gastrostomía extrayendo tricobezoar gástrico, que en el estudio anatomopatológico pesó 1033,8 g y midió 31,5 x 18 cm. en sus dimensiones mayores. Estos casos son sumamente raros. En nuestro caso el tricobezoar alcanzó el tamaño más grande de los hasta ahora reportados. Contradictoriamente la sintomatología fue mínima.

A trichobezoar is a compact mass of hair that is usually stored in the stomach. It is common in young women who eat their own hair (trichophagia) due to psychological disorders. We present the case of a 13-year-old teenager who had been brought in an emergency for having persistent abdominal pain for 3 days. Abdominal ultrasound was suggestive of trichobezoar, a finding that was confirmed by upper gastrointestinal endoscopy. For two years before her admission, she had chewed her hair and consumed it, which she associated with her brother's trip abroad and the separation of her parents. On the ninth day, an exploratory laparotomy with gastrostomy was performed, extracting gastric trichobezoar, which in the anatomopathological study weighed 1033.8 g. and measured 31.5 x 18 cm. in its larger dimensions. These cases are extremely rare. In our case, the trichobezoar reached the largest size of those reported so far. Contradictorily, the symptoms were minimal.

El "barbering": un problema frecuente en ratones de laboratorio / Barbering: a frequent problem in laboratory mice / O comportamento de barbeiro: um problema frequente in camundongos de laboratório

Resumen El barbering es una conducta que consiste en halar o recortar el pelo y en algunos casos incluso ingerirlo. Este comportamiento ha sido reportado tanto en el ser humano como en otras especies, no obstante, su presentación en ratones de laboratorio es frecuente en algunos linajes, genera impacto sobre su bienestar y la fiabilidad de los resultados de investigación, siendo un problema destacado en los bioterios de ratones. Al realizar una revisión de literatura disponible sobre esta condición, se identifican múltiples causas, entre estas, deficiencias en el enriquecimiento ambiental, alteraciones de manejo, estrés, conductas aprendidas, ansiedad y dominancia, así como causas genéticas haciendo que algunas cepas, linajes y modelos genéticamente modificados sean más susceptibles al barbering y ayudando a facilitar la comprensión de rutas metabólicas involucradas en esta condición. La etiología múltiple del barbering permite abordar opciones de manejo orientadas hacia la intervención ambiental y algunas estrategias farmacológicas que podrían ser útiles para reducir su incidencia y los problemas de salud y desempeño reproductivo asociados.

Abstract Barbering is a behavior that consists of pulling, cutting, or even ingesting hair. This behavior has been reported in humans or other animals; nevertheless, its presentation in laboratory mice is common in some mice strains, affects these animals' welfare, and results of research studies being a problem in mice facilities. After reviewing the available literature on this condition, multiple causes are identified, including deficiencies in environmental enrichment, management alterations, stress, learned behaviors, anxiety, and dominance, as well as genetic causes, making some strains, lineages, and genetically modified models more susceptible to barbering and helping to facilitate the understanding of metabolic pathways involved in this condition. The multiple etiology of barbering makes it possible to approach management options oriented towards environmental intervention and some pharmacological strategies that could be useful to reduce its incidence and associated reproductive health and performance problems.

Resumo O comportamento de barbeiro consiste em puxar ou aparar o pelo e, em alguns casos, até mesmo ingeri-lo. Esse comportamento tem sido relatado tanto em humanos quanto em outras espécies, entretanto, sua apresentação em camundongos de laboratório é frequente em algumas linhagens, gerando impacto no seu bem-estar e na confiabilidade dos resultados das pesquisas, sendo um problema de destaque nos biotérios. Ao realizar uma revisão da literatura disponível sobre esta condição, múltiplas causas foram identificadas, incluindo deficiências no enriquecimento ambiental, alterações de manejo, estresse, comportamentos aprendidos, ansiedade e dominância, além de causas genéticas, tornando mais suscetíveis à barbearia à algumas linhagens e modelos geneticamente modificados e ajudando a facilitar o entendimento das vias metabólicas envolvidas nessa condição. A múltipla etiologia deste comportamento de barbeiro permite abordar opções de gestão orientadas para a intervenção ambiental e algumas estratégias farmacológicas que podem ser úteis para reduzir a sua incidência e os problemas de saúde e desempenho reprodutivos associados.

Tricobezoar gástrico y gastroduodenal en pediatría. Reporte de 2 casos e implicaciones quirúrgicas / Gastric and Gastroduodenal Trichobezoar in Pediatrics. Report of 2 Cases and surgical Implications

Resumen El tricobezoar es una variedad de los bezoarios cuya característica es la presencia de una masa compacta constituida por material no digerible, principalmente cabellos y otros componentes. Presentación de casos: Se presentan 2 casos de pacientes de 4 y 9 años, quienes ingresaron por emergencias, con evolución clínica crónica caracterizada por una masa abdominal palpable, pérdida de peso progresiva asociada a tricofagia-tricotilomania, con presunción diagnóstica de ingreso de bezoar gástrico. La ecografía abdominal en ambos pacientes reportó imagen ecorefringente en su interior que produce sombra acústica, compatible con probable tricobezoar, diagnóstico reforzado por tomografía computarizada con contraste. Dada la imposibilidad de extracción vía endoscópica por el gran tamaño, se optó por realizar laparotomía exploratoria, extrayéndose en los 2 casos la masa en bloque completa a través de una gastrotomía. Las pacientes presentaron una evolución favorable, recibiendo alimentación parenteral por 5 días y luego ingesta oral con buena tolerancia, dando de alta hospitalaria a su 9° y 10° día postoperatorio, con seguimiento interdisciplinario y apoyo psicológico. Debe considerarse como primera posibilidad diagnóstica el tricobezoar ante presencia de tricotilomanía, compromiso afectivo, retraso psicomotor, clínica intestinal. En estos casos el manejo neuropsiquiátrico postquirúrgico es fundamental a fin de reducir el riesgo de recurrencias.

Abstract The trichobezoar is a variety of bezoars whose characteristic is the presence of a compact mass made up of indigestible material, mainly hair and other components. Presentation of cases: We present 2 cases of patients aged 4 and 9 years, who were admitted to an emergency room, with symptoms of chronic evolution characterized by a palpable abdominal mass, progressive weight loss associated with trichophagia-trichotillomania, with presumed diagnosis of gastric bezoar admission. The abdominal ultrasound in both reported an echorefringent image inside that produces an acoustic shadow, compatible with a probable trichobezoar, a diagnosis reinforced by contrast-enhanced computed tomography. Given the impossibility of endoscopic extraction due to the large size, it was decided to perform exploratory laparotomy, extracting the complete en-bloc mass through a gastrotomy in both cases. The patients presented a favorable evolution, receiving parenteral feeding for 5 days and then oral ingestion with good tolerance, being discharged from hospital on their 9th and 10th postoperative day with interdisciplinary follow-up and psychological support. There is a high suspicion of trichobezoar in the presence of trichotillomania, affective compromise, psychomotor retardation, intestinal symptoms and postsurgical neuropsychiatric management is essential in order to reduce the risk of recurrence.

Tricobezoar gástrico: reporte de un caso y revisión de la literatura

El bezoar gástrico es una tumoración por cuerpo extraño que resulta de la acumulación de material no digerible en el estómago. El tricobezoar es uno de ellos y frecuentemente se presenta en la población femenina joven que padece trastornos psiquiátricos. La presentación del bezoar gástrico es insidiosa e inespecífica, teniendo un curso inicialmente asintomático por años, hasta que alcanza un tamaño que evidencia síntomas. El método diagnóstico de elección es la endoscopía, ya que permite visualizar el bezoar y plantear el tratamiento. El abordaje terapéutico estará determinado por el tipo, tamaño y consistencia de este; sin embargo, la resolución quirúrgica es la de elección, la cual siempre debe estar asociada a tratamiento psiquiátrico para prevenir la recurrencia del cuadro. Se presenta el caso de una paciente de 19 años con el diagnóstico de tricobezoar gástrico, asociado a tricotilomanía y tricofagia como patologías de fondo, y se realiza una revisión de la literatura.

A gastric bezoar is a foreign body tumor that results from the accumulation of indigestible material in the stomach. The trichobezoar is one of them and frequently occurs in the young female population suffering from psychiatric disorders. The presentation of the gastric bezoar is insidious and nonspecific, having an initially asymptomatic course for years, until it reaches a size that shows symptoms. The diagnostic method of choice is endoscopy since it allows the bezoar to be visualized and propose the treatment. The therapeutic approach will be determined by its type, size, and consistency; however, surgical resolution is the one of choice, which must always be associated with psychiatric treatment to prevent recurrence of the condition. The case of a 19-year-old patient with a diagnosis of gastric trichobezoar, associated with trichotillomania and trichophagia as underlying pathologies is presented, and a literature review is carried out.

El largo camino de la tricotilomania al síndrome de Rapunzel / The Long Road from Trichotillomania to Rapunzel Syndrome

Introducción: La tricotilomania, la tricofagia y el tricobezoar son afecciones entrelazadas e interconectadas. Estas deben tenerse en cuenta especialmente en féminas jóvenes que a menudo tienen una enfermedad psiquiátrica o trastorno social subyacente y es posible que la historia sea difícil de obtener. Objetivo: Presentar un caso portador de tricobezoar gástrico, por su baja incidencia en nuestro medio y a nivel mundial. Caso clínico: Paciente femenina de 12 años de edad, con antecedentes de retraso psicomotor, sin seguimiento o tratamiento previo. Acudió a consulta traída por su madre que aduce aumento de volumen en hemiabdomen superior de 5 años de evolución con pérdida de peso insidiosa. Los estudios radiológicos revelaron un tricobezoar gástrico que se corrobora, asociado a un síndrome de Rapunzel, en la laparotomía exploratoria. La paciente evolucionó satisfactoriamente y es seguida por grupo multidisciplinario. Conclusiones: Los bezoares gástricos son entidades poco frecuentes. Para el diagnóstico es necesario un alto índice de sospecha de conjunto con estudios radiográficos y/o endoscópicos. El tratamiento estará determinado por el tipo de bezoar, tamaño y consistencia. La opción quirúrgica es la más usada, con el consecuente seguimiento interdisciplinario para evitar recurrencias(AU)

Introduction: Trichotillomania, trichophagia and trichobezoar are intertwined and interconnected conditions. These should be considered, especially in young women who often have an underlying psychiatric illness or social disorder and possibly a history difficult to get. Objective: To report a case of gastric trichobezoar due to its low incidence in our environment and worldwide. Clinical case report: We report the case of a 12-year-old female patient with a history of psychomotor retardation, with no prior follow-up or treatment, who came to consult with her mother, complaining of increased volume in the upper abdomen, of 5 years of evolution with malignant weight loss. Radiological studies reveal a gastric trichobezoar that is corroborated, associated with Rapunzel syndrome, in the exploratory laparotomy. The patient evolved satisfactorily and a multidisciplinary group assisted her. Conclusions: Gastric bezoars are rare entities. Diagnosis requires high level of suspicion together with radiographic and/or endoscopic studies. The treatment will be determined by the type of bezoar, size and consistency. The surgical option is the most used, requiring interdisciplinary follow-up to avoid recurrences(AU)

Isolated hair loss on the eyebrow: five cases with trichoscopic features

Abstract Alopecia areta (AA) and trichotillomania (TTM) are common causes for hair loss on the eyebrows. Yellow dots, vellus hairs, anisotrichosis, empty follicular openings, and black dots were observed in the present study's patients with AA. Split hairs, question mark hairs, broken hairs, flame hairs, black dots, hairs with different lengths, and hemorrhagic areas were found in the patients with TTM. Trichoscopy is a very useful and helpful technic in distinguishing AA and TTM on the eyebrows.

Síndrome de Rapunzel más que una leyenda infantil: caso clínico pediátrico / Syndrome of Rapunzel more than an infantile legend: pediatric clinical case

RESUMEN Fundamento: el tricobezoar es poco frecuente y el síndrome de Rapunzel es muy raro, pero se debe tener presente como diagnóstico diferencial en niñas con trastornos psicológicos y tricofagia, que refieran o no manifestaciones gastrointestinales. Objetivo: presentar el caso de una escolar de nueve años con historia de tricofagia que acude a servicio de Gastroenterología por presentar tumor palpable localizado en epigastrio. Presentación del caso: paciente de nueve años, femenina, que presenta tricotilomanía y tricofagia desde los dos años de vida, que acude por tumor palpable en epigastrio, sin otras manifestaciones clínicas. Conclusiones: el diagnóstico temprano del tricobezoar evita las complicaciones que pueden ser graves y se recomienda un manejo multidisciplinario con el servicio de psiquiatría para controlar la enfermedad de base. La evolución de la paciente fue favorable.

ABSTRACT Background: the tricobezoar is not very frequent and the syndrome of Rapunzel is very strange, but it should be taken into account as differential diagnosis in girls with psychological dysfunctions and trichophagia that refer or not gastrointestinal manifestations. Objective: to present the case of a nine year-old school girl with trichophagia history that goes to service of Gastroenterology to present palpable tumor in epigastrium. Case report: patient of nine years, feminine that presents trichotollomania and trichophagia since she was two years old, she goes for palpable tumor in lengthened epigastrium, without other clinical manifestations. Conclusions: early diagnosis of trichobezoar avoids complications that can be serious, and multidisciplinary management with the psychiatry service is recommended to control the underlying disease. The evolution of the patient was favorable.

Gastric trichobezoar and Rapunzel syndrome: a case report

Giant gastric trichobezoars are unusual form of foreign body found in stomach of mostly young adolescent females which may lead to morbidity and high mortality 30%, if goes unnoticed. These females have history of trichophagia or trichotillomania. This report is of a 12- year old young adolescent female presented with epigastric pain and mass. An exploratory laparotomy with anterior gastrotomy was performed and a giant trichobezoar with a very large tail of 2.5 ft was removed, weighing 2.52 kg. She recovered well and was discharged on 7th post-operative day. Patient was advised for psychiatric follow up.

Trichotillomania is more related to Tourette disorder than to obsessive-compulsive disorder

Objective: Trichotillomania (TTM) is characterized by the pulling out of one's hair. TTM was classified as an impulse control disorder in DSM-IV, but is now classified in the obsessive-compulsive related disorders section of DSM-5. Classification for TTM remains an open question, especially considering its impact on treatment of the disorder. In this review, we questioned the relation of TTM to tic disorder and obsessive-compulsive disorder (OCD). Method: We reviewed relevant MEDLINE-indexed articles on clinical, neuropsychological, neurobiological, and therapeutic aspects of trichotillomania, OCD, and tic disorders. Results: Our review found a closer relationship between TTM and tic disorder from neurobiological (especially imaging) and therapeutic standpoints. Conclusion: We sought to challenge the DSM-5 classification of TTM and to compare TTM with both OCD and tic disorder. Some discrepancies between TTM and tic disorders notwithstanding, several arguments are in favor of a closer relationship between these two disorders than between TTM and OCD, especially when considering implications for therapy. This consideration is essential for patients.

Tricotilomanía de nueva aparición durante el tratamiento con fármacos estimulantes. A propósito de dos casos clínicos pediátricos / New-onset trichotillomania during treatment with stimulant drugs. About two pediatric clinical cases

Los fármacos estimulantes se usan, habitualmente, en la población pediátrica para tratar el trastorno por déficit de atención e hiperactividad, y sus efectos secundarios están bien descritos. Sin embargo, la tricotilomanía no aparece como uno de ellos. En la literatura, hay algunos casos publicados de tricotilomanía en relación con la administración de metilfenidato y dextroanfetamina. Se presentan dos casos de tricotilomanía de nueva aparición en niños en seguimiento en nuestro Centro por déficit de atención e hiperactividad y en tratamiento con fármacos psicoestimulantes (metilfenidato y lisdexanfetamina), como probable efecto adverso de estos.

Stimulant drugs are commonly used in pediatric population in the treatment of attention deficit hyperactivity disorder, and their side effects are well described, however trichotillomania does not appear as one of them. In the literature we found some published cases of trichotillomania in relation to methylphenidate and dextroamphetamine. We present two cases of new-onset trichotillomania in children followed up in our center by attention deficit hyperactivity disorder and treated with psychostimulant drugs (methylphenidate and lisdexamfetamine), as a probable adverse effect of this treatment

Tricobezoar gastroduodenal en la edad escolar: Caso clínico / Gastroduodenal Trichobezoar in school age: Case report

Los bezoares son acumulaciones de sustancias orgánicas en el tracto gastrointestinal. El tricobezoar hace referencia a la acumulación de cabello, por lo general, en el estómago e incluso más allá de este (síndrome de Rapunzel). La incidencia de esta enfermedad es extremadamente rara; el 90 % de los casos se presentan en las mujeres, y el 80 % de estos ocurren en menores de 30 años y se encuentran relacionados con conductas de ansiedad y trastornos psiquiátricos. El abordaje integral en estos pacientes es fundamental, junto con el seguimiento interdisciplinario, para tratar las causas psicoemocionales subyacentes y realizar una adecuada psicoeducación con los pacientes y sus familiares, para disminuir el riesgo de posibles recidivas. Se presenta el caso clínico de una paciente en edad escolar, con antecedente de maltrato físico por parte de su padrastro y posteriores conductas ansiosas (tricotilomanía y tricofagia).

Bezoar is an accumulation of organic substances in the gastrointestinal tract; trichobezoar refers to the accumulation of hair usually in the stomach and even beyond it (Rapunzel syndrome). The incidence of this condition is extremely rare, 90 % of cases occur in women, and 80 % of these occur in those under 30 years of age and are related to anxiety behaviors and psychiatric disorders. The integral approach in these patients is fundamental, together with the interdisciplinary follow-up, treating the underlying psycho-emotional causes and adequate psychoeducation with the patients and their relatives, in order to reduce the risk of possible recurrences. We present the clinical case of a patient of school age, with a history of physical abuse by her stepfather and subsequent anxious behaviors (trichotillomania and trichophagia).

The cause of abdominal mass in a child with celiac disease: Rapunzel syndrome. A case report

ABSTRACT CONTEXT: Rapunzel syndrome is a rare form of gastric trichobezoar that develops through outstretching of the bezoar from the stomach to the intestine. CASE REPORT: A 12-year-old girl who had been diagnosed with celiac disease six years earlier was brought to the department of pediatric gastroenterology because of abdominal distension. A palpable mass was detected. A trichobezoar that stretched to the small intestine was removed surgically. The patient was diagnosed as having anxiety and depressive disorder, and treatment started. Following the treatment, her previous trichophagia completely disappeared. CONCLUSION: Presence of trichobezoar should be kept in mind, especially when young girls who have psychiatric problems suffer from gastrointestinal symptoms.

Trichobezoars as a cause of intestinal obstruction in a young female

A bezoar is a mass found trapped in the gastrointestinal system, though it can occur in other locations. There are various types of bezoars including food boluses, lactobezoar, pharmacobezoar, phytobezoar, diospyrobezoar and trichobezoar. They are common in children and adolescents, and 90% of the patients are women. Complications may manifest as gastric outlet obstruction or bleeding and intestinal obstruction. CT is the preferred image modality for the evaluation of suspected trichobezoars in order to characterize the size, location, presence and level of obstruction and complications such as ischemia or perforation. Here authors present a case to stress the importance of considering trichobezoars as one of the causes for intestinal obstruction in young females.

A rare presentation of Rapunzel syndrome with multiple small bowel intussusceptions

Rapunzel syndrome is caused by gastric trichobezoar with extended tail and small bowel obstruction. Patients with gastric trichobezoar can be asymptomatic until the bezoar increases in size. We report a case of a girl who visited the emergency department with abdominal pain. She was finally diagnosed with Rapunzel syndrome that causes multiple small bowel intussusceptions associated with trichophagia. Surgery was needed to reduce the multiple intussusceptions, and to remove the large trichobezoar. This case highlights to consider the possibility of Rapunzel syndrome when diagnosing the main cause of intussusceptions.

N-acetylcysteine in dermatology

N-acetylcysteine is a mucolytic drug which is commonly used as an antidote for acetaminophen toxicity. It is a thiol compound, which acts as a donor of cysteine, leading to replenishment of glutathione and thus acts as an antioxidant. It also has anti-inflammatory effects, alters the levels of neurotransmitters, inhibits proliferation of fibroblasts and keratinocytes and causes vasodilatation. Due to these actions, n-acetylcysteine has found use in several dermatologic conditions in systemic and topical form. The drug has been used as an adjuvant in the management of conditions such as toxic epidermal necrolysis, drug hypersensitivity syndrome, trichotillomania, skin picking disorders and onychotillomania, ichthyoses, contact dermatitis, atopic dermatitis, melasma, pseudoporphyria, connective tissue diseases, wound healing and alopecia. It also has a role in protection from radiation-induced skin damage including photo-ageing, photocarcinogenesis and radiation dermatitis. Most indications in dermatology are supported by case reports, small case series and small trials. Higher quality of evidence is needed for its wider use. The drug is cheap and is generally safe with few adverse effects. Thus a greater role is possible for use of n-acetylcysteine in various skin conditions. This review explores the various uses of n-acetylcysteine in the field of dermatology, the evidence supporting the same, the possible mechanisms of action and the adverse effects of the drug.

Síndrome de Rapunzel con doble tricobezoar simultáneo en una adolescente: Reporte de caso / Rapunzel syndrome with double simultaneous trichobezoar in a teenager: Clinical Case Report

Resumen: Introducción: Los tricobezoares son acúmulos intraluminales de cabello ingerido. El síndrome de Rapunzel hace referencia a la presencia de los tricobezoares gástricos que se extienden al intestino delgado, sumados a la tricotilomanía y tricofagia, los cuales ocurren preferentemente en pacientes psiquiátricos en edad pediátrica. Objetivo: Analizar la aparición y manejo de este síndrome, propor cionando de igual manera datos acerca del entorno familiar y psicoemocional para que, por medio del análisis de los mismos, en un futuro se pueda identificar el riesgo en pacientes con circunstancias similares. Caso clínico: Paciente femenina de 14 años con antecedentes de tricotilomanía y tricofagia de dos años de evolución, que consultó por cuadro de dolor epigástrico asociado a sensación de plenitud posprandial, náuseas y pérdida de peso. Al examen destacaban áreas alopécicas en el cuero cabelludo y a la palpación abdominal se identificó un plastrón cuyo contorno parecía corresponder a los límites gástricos. En los estudios de imágenes se encontró una ocupación gástrica por bezoar. Con la laparotomía más gastrostomía se identificaron dos tricobezoares simultáneos en estómago y duodeno, que fueron resueltos quirúrgicamente y la paciente fue manejada con abordaje psicoemocional. Conclusión: El síndrome de Rapunzel, lejos de ser meramente una entidad quirúrgica, requiere un apoyo psicoemocional para prevenir su recurrencia y limitar su severidad.

Resumen: Introduction: Trichobezoars are an intraluminal accumulation of ingested hair. The Rapunzel syndrome refers to the presence of gastric trichobezoars which extend to the small intestine together with trichotillomania and trichophagia, that occur predominantly in psychiatric patients of pediatric age. Objective: To analyze the clinical course and resolution of this syndrome in a case report. Likewise, we provide information about the family environment and psycho-emotional context of the patients and help the reader identify similar circumstances in their clinical practice. Case report: Female 14-year-old patient with history of trichotillomania and trichophagia of two years of evolution, who consulted for epigastric pain associated with weight loss, nausea, and postprandial fullness. During the physical examination, the patient was found to have bald patches in the scalp along with a palpable mass that seemed to be confined to the gastric limits. Imaging studies revealed gastric occupation due to a bezoar formation. The patient was treated surgically with laparotomy and gastrostomy, and two simultaneous trichobezoars were removed from the patient´s stomach and duodenum, the patient also underwent psycho-emotional professional counseling. Conclusion: Rapunzel´s syndrome, far for being a merely surgical entity, also requires psychoemotional assessment to prevent it recurrence and limit its severity.

A Pilot Examination of Oxidative Stress in Trichotillomania

OBJECTIVE: Trichotillomania is a relatively common illness whose neurobiology is poorly understood. One treatment for adult trichotillomania, n-acetyl cysteine (NAC), has antioxidative properties, as well as effects on central glutamatergic transmission. Preclinical models suggest that excessive oxidative stress may be involved in its pathophysiology. METHODS: Adults with trichotillomania provided a blood sample for analysis of compounds that may be influenced by oxidative stress [glutathione, angiotensin II, ferritin, iron, glucose, insulin and insulin growth factor 1 (IGF1), and hepcidin]. Participants were examined on symptom severity, disability, and impulsivity. The number of participants with out-of-reference range oxidative stress measures were compared against the null distribution. Correlations between oxidative stress markers and clinical measures were examined. RESULTS: Of 14 participants (mean age 31.2 years; 92.9% female), 35.7% (n=5) had total glutathione levels below the reference range (p= 0.041). Other oxidative stress measures did not have significant proportions outside the reference ranges. Lower levels of glutathione correlated significantly with higher motor impulsiveness (Barratt Impulsiveness Scale sub-score) (r=0.97, p=0.001). CONCLUSION: A third of patients with trichotillomania had low levels of glutathione, and lower levels of glutathione correlated significantly with higher motor impulsiveness. Because NAC is a precursor for cysteine, and cysteine is a rate limiting step for glutathione production, these results may shed light on the mechanisms through which NAC can have beneficial effects for impulsive symptoms. Confirmation of these results requires a suitable larger follow-up study, including an internal normative control group.

Trichotillomania: a good response to treatment with N-acetylcysteine

Abstract: Trichotillomania is considered a behavioral disorder and is characterized by the recurring habit of pulling one's hair, resulting in secondary alopecia. It affects 1% of the adult population, and 2 to 4.4% of psychiatric patients meet the diagnostic criteria. It can occur at any age and is more prevalent in adolescents and females. Its occurrence in childhood is not uncommon and tends to have a more favorable clinical course. The scalp, eyebrows and eyelashes are the most commonly affected sites. Glutamate modulating agents, such as N-acetylcysteine, have been shown to be a promising treatment. N-acetylcysteine acts by reducing oxidative stress and normalizing glutaminergic transmission. In this paper, we report a case of trichotillomania with an excellent response to N-acetylcysteine.