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1.
São Paulo med. j ; 140(5): 723-733, Sept.-Oct. 2022. tab, graf
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1410217

RESUMEN

ABSTRACT BACKGROUND: The number of bariatric surgeries performed worldwide is growing. Among the main short, medium or long-term complications after surgery are nutritional deficiencies. Many of these, such as those of Zn, Cu and vitamins A, B1, B3, B6 and B12, are manifested by dermatological lesions before potentially fatal systemic disorders occur. OBJECTIVE: To identify the main dermatological manifestations associated with nutritional deficiencies after bariatric surgery, and the associated variables. DESIGN AND SETTING: Integrative literature review carried out at a public university in Brazil. METHODS: This was a case report and a review of health research portals and databases of national and international biomedical journals, without publication date limitation. The descriptors used for searches followed the ideal methodology for each database/search portal: "bariatric surgery", "skin", "skin disease", "skin manifestation", "deficiency disease" and "malnutrition". RESULTS: A total of 59 articles were selected, among which 23 were review articles or articles that addressed specific dermatological manifestations. The other 36 articles described 41 cases, which were organized into a table with the clinical variables. CONCLUSIONS: Although nutritional deficiencies are expected as complications after bariatric surgery, few articles relating them to their dermatological manifestations were found. It is important to recognize skin changes caused by nutritional deficiencies in patients treated via bariatric surgery, as these may occur before systemic complications appear and are easier to diagnose when the patient does not have any systemic symptoms yet. However, there is generally a delay between the appearance of skin lesions and making the diagnosis of nutritional deficiency.

2.
Rev. cuba. pediatr ; 92(2): e923, abr.-jun. 2020. graf
Artículo en Español | LILACS, CUMED | ID: biblio-1126753

RESUMEN

Introducción: La acrodermatitis enteropática es una dermatosis nutricional heredada o adquirida por deficiencia de zinc que clínicamente se caracteriza por eritema, escamocostras y erosiones, especialmente en la región perioral, en zonas acras y en la región anogenital. Además de una ingesta oral inadecuada, hay causas secundarias de esta deficiencia nutricional. Objetivo: Referir una dermatosis nutricional secundaria infrecuente en lactante femenina. Presentación de caso: Pequeñita de 6 meses de edad, fototipo III, alimentada con lactancia materna exclusiva, que acude a consulta especializada de dermatología en el hospital William Soler por cuadro clínico de 3 meses de evolución caracterizado por lesiones eritematocostrosas en regiónes acrales y periorificiales. Los padres refirieron lactancia materna exclusiva. Se realizaron complementarios Conclusiones: Las deficiencias de vitaminas y oligoelementos pueden ocasionar un amplio rango de hallazgos mucocutaneos. En el caso presentado diagnosticado como acrodermatitis enteropática adquirida, hay un aporte inadecuado de zinc en la leche materna(AU)


Introduction: Acrodermatitis enteropathica is an inherited or acquired nutritional dermatosis by zinc deficiency that is clinically characterized by erythema, squamous crusts and erosions, especially in the perioral region, in acral areas and in the anogenital region. In addition to inadequate oral intake, there are secondary causes of this nutritional deficiency. Objective: To refer a nutritional dermatosis uncommon in female infants. Case presentation: 6-months-old baby girl, skin phototype III, fed with exclusive breastfeeding whom was attended in the specialized consultation in Dermatology of William Soler Pediatric Hospital due to clinical framework of 3 months of evolution characterized by eritemato crusty lesions in acral and periorifices´ regions. Parents commented on exclusive breastfeeding. There were made complementary blood tests. Conclusions: The deficiencies of vitamins and trace elements may cause a wide range of mucocutaneous findings. In the presented case diagnosed as adquired acrodermatitis enteropathica, there is an inadequate intake of zinc in breast milk(AU)


Asunto(s)
Humanos , Femenino , Lactante , Acrodermatitis/diagnóstico , Acrodermatitis/epidemiología
3.
Bol. méd. Hosp. Infant. Méx ; 74(4): 295-300, jul.-ago. 2017. tab, graf
Artículo en Español | LILACS | ID: biblio-888629

RESUMEN

Resumen: Introducción: La acrodermatitis enteropática es una enfermedad de baja incidencia que ocurre por deficiencia de zinc; puede ser hereditaria o adquirida. Se caracteriza por dermatitis acral, alopecia, diarrea y problemas de crecimiento. La afección dermatológica puede simular una infección micótica cutánea u otras enfermedades de la piel relacionadas con patógenos. Caso clínico: Se informa el caso de una paciente de sexo femenino de 7 meses de edad, que fue enviada al Centro Médico Nacional 20 de Noviembre por sospecha de inmunodeficiencia y micosis cutánea. Inició con un cuadro clínico caracterizado por dermatosis diseminada a cabeza, tronco y región genital, por lo que recibió tratamiento inicial con un antifúngico y antibióticos de amplio espectro, sin presentar mejoría. A su ingreso se descartaron inmunodeficiencia e infección micótica. Se sospechó acrodermatitis enteropática, corroborándose el diagnóstico al encontrar niveles séricos de zinc disminuidos. La paciente presentó mejoría inmediata tras el inicio del tratamiento con zinc por vía oral. Conclusiones: El diagnóstico diferencial de acrodermatitis enteropática es amplio e incluye infecciones cutáneas, por lo que el reconocimiento temprano de las características de las lesiones favorece la sospecha, el diagnóstico y el tratamiento adecuado.


Abstract: Introduction: Acrodermatitis enteropathica is a low-incidence disease due to inherited or acquired zinc deficiency. It is characterized by acral dermatitis, alopecia, diarrhea and growth retardation. The dermatological condition may mimic a cutaneous fungal infection or other pathogen-related skin diseases. Case report: We report the case of a female patient of 7 months of age, who was sent to Centro Médico Nacional 20 de Noviembre for suspicion of immunodeficiency and cutaneous mycosis. Her condition began with dermatosis disseminated to the head, trunk and genital region; initial treatment with antifungal and broad spectrum antibiotics was given, without improvement. Upon admission, immunodeficiency and fungal infection were discarded. Acrodermatitis enteropathica was suspected, and corroborated later by low serum zinc levels. Immediately after the start of oral treatment with zinc, the patient showed improvement. Conclusions: There are multiple differential diagnoses of acrodermatitis enteropathica, which includes cutaneous infections. Therefore, the early recognition of the characteristic lesions favors suspicion, diagnosis and appropriate treatment.

4.
Malaysian Journal of Dermatology ; : 69-71, 2017.
Artículo en Inglés | WPRIM | ID: wpr-731982

RESUMEN

Acrodermatitis enteropathica (AE) is a rare genetic autosomal recessive disorder characterised byperiorificial inflammatory rash, diarrhoea and hair loss. Hereby, we report 3 cases of acrodermatitisenteropathica.

5.
Allergy, Asthma & Respiratory Disease ; : 361-365, 2017.
Artículo en Coreano | WPRIM | ID: wpr-114703

RESUMEN

Acrodematitis enteropathica is an uncommon disease due to zinc deficiency which is characterized by perioral and acral dermatitis, alopecia, and diarrhea. This disease typically presents in infants, either due to an autosomal recessive genetic disorder or after cessation of breastfeeding. Acquired adult-onset acrodermatitis enteropathica has rarely been reported so far. We report a case of a 38-year-old man who had a history of chronic alcohol intake and presented with severe acrodermatitis enteropathica. The patient was successfully managed with zinc supplement and supportive care.


Asunto(s)
Adulto , Humanos , Lactante , Acrodermatitis , Alopecia , Lactancia Materna , Dermatitis , Diarrea , Zinc
6.
Rev. chil. pediatr ; 86(1): 52-55, feb. 2015. ilus
Artículo en Español | LILACS | ID: lil-745611

RESUMEN

Introducción: Las manifestaciones dermatológicas en el momento del diagnóstico de fibrosis quística son infrecuentes. Objetivo: Describir el caso de una lactante con una presentación dermatológica no habitual de fibrosis quística. Caso Clínico: Lactante que a los 2 meses de edad presenta un mal incremento pondoestatural. A los 4 meses presenta exantema cutáneo, edema y pelo gris. Dentro de su estudio se evidencia hipoproteinemia y anemia, y se confirma el diagnóstico de fibrosis quística mediante el estudio genético. Las alteraciones dermatológicas se revirtieron completamente tras la instauración de la terapia de reemplazo con enzimas pancreáticas. Este es el segundo caso reportado de pelo gris en niños con esta enfermedad. Conclusión: En niños con malnutrición que desarrollan alteraciones de la piel y fanéreos debemos sospechar enfermedades metabólicas como fibrosis quística.


Introduction: Cutaneous manifestations at the time of CF diagnosis are rare. Objective: To describe the case of an infant with an unusual cutaneous presentation of cystic fibrosis. Case report: The case is presented of an infant with delayed physical growth at two months, and at the age of four months, presented with a skin rash and gray hair. Tests revealed the presence of hypoproteinemia and anemia, with the diagnosis of Cystic Fibrosis being confirmed by genetic testing. The rash was completely resolved after pancreatic enzyme replacement therapy. This is the second gray hair case reported in children with this disease. Conclusion Metabolic diseases such as cystic fibrosis should be suspected in malnourished children who develop skin disorders.


Asunto(s)
Humanos , Femenino , Lactante , Zinc/deficiencia , Acrodermatitis/etiología , Color del Cabello , Fibrosis Quística/diagnóstico , Terapia de Reemplazo Enzimático/métodos , Pruebas Genéticas/métodos , Fibrosis Quística/complicaciones , Hipoproteinemia/etiología , Anemia/etiología
7.
Bol. méd. Hosp. Infant. Méx ; 69(6): 475-480, nov.-dic. 2012. ilus
Artículo en Español | LILACS | ID: lil-701173

RESUMEN

Introducción. La acrodermatitis enteropática es una patología rara, pero de fácil manejo y gran relevancia clínica. Debe ser diagnosticada de manera adecuada y oportuna. Se presenta en lactantes con la siguiente triada clínica: dermatitis acral, diarrea y alopecia. Se deberá iniciar tratamiento con zinc, incluso desde un primer nivel de atención, ya que la respuesta clínica es inmediata y sin secuelas. Caso clínico. Se reporta el caso de un lactante mayor con desnutrición crónica, talla baja y retraso psicomotor, así como grandes lesiones cutáneas descamativas, simétricas y diseminadas, y alopecia total, que ingresó al Hospital Regional de la Huasteca, en Huejutla, Hidalgo, México. Se sospechó de manera clínica en acrodermatitis enteropática, por lo que se determinaron los niveles séricos de zinc y se realizó una biopsia cutánea. Se obtuvo mejoría clínica desde las primeras dos semanas de tratamiento. Conclusiones. El tratamiento con sulfato de zinc a dosis de 2-5 mg/ kg/día presenta efectos clínicos inmediatos, con remisión completa de la sintomatología.


Background. Acrodermatitis enteropathica is a rare but easy to manage condition but with great clinical relevance. The condition must be diagnosed properly and timely. We present an infant with the following clinical triad: acral dermatitis, diarrhea and alopecia. Zinc treatment should be initiated, even from a primary care level. Clinical response is immediate and without sequelae. Case report. We present the case of an infant with chronic malnutrition, short stature, psychomotor retardation and large symmetrical scaly skin lesions with disseminated alopecia totalis. The patient was admitted to the Hospital Regional de la Huasteca, Huejutla, Hidalgo. Acrodermatitis enteropathica was suspected in the clinic and serum zinc and skin biopsy were carried out. Clinical improvement was obtained from the first 2 weeks oftreatment. Conclusions. Treatment initiation with zinc sulfate at a dose of 2-5 mg/kg/day has immediate clinical implications with complete symptom remission.

8.
Annals of Dermatology ; : S88-S90, 2011.
Artículo en Inglés | WPRIM | ID: wpr-224515

RESUMEN

Acrodermatitis enteropathica is classified as a congenital autosomal recessive type and an acquired transient type. This disease manifests as acral and periorificial dermatitis, alopecia, intractable diarrhea, and failure to thrive. Whereas the autosomal hereditary type is caused by malabsorption of zinc in the intestine, the acquired type is caused by low nutritional support or decreased peripheral release of zinc from blood. We experienced a case of a 5-month old, breast feeding, full-term female presenting with only acral bullous dermatitis without diarrhea, periorificial dermatitis and an abnormal serum zinc level.


Asunto(s)
Femenino , Humanos , Acrodermatitis , Alopecia , Lactancia Materna , Dermatitis , Diarrea , Insuficiencia de Crecimiento , Pie , Mano , Intestinos , Apoyo Nutricional , Zinc
9.
Annals of Dermatology ; : S326-S328, 2011.
Artículo en Inglés | WPRIM | ID: wpr-165619

RESUMEN

Acrodermatitis enteropathica (AE) is an uncommon autosomal recessive genetic disorder of zinc malabsorption. The acquired form may be associated with inadequate intake, impaired absorption, and increased excretion of zinc. Those afflicted present with diarrhea, stomatitis, psychiatric symptoms, non-scarring alopecia, and nail dystrophy accompanied by erythematous which appears as scaly patches with erosion vesicles and pustules mostly affecting the extremities, perineal, and periorificial areas. Due to the variable findings of most case reports, the clinical and histopathological features of AE are often regarded as non-specific. We report an unusual case of bullous AE secondary to total parenteral nutrition for the treatment of acute pancreatitis occurring in a six-year-old male with acute lymphocytic leukemia who underwent chemotherapy. He presented with periorificial, reddish, eroded bullae with multiple vesicles and blisters on his fingers, toes, and buttock, showing necrotic keratinocytes with multiple intraepidermal vesicles and perivascular infiltration with predominant lymphocytes and few neutrophils within the dermis. To the best of our knowledge, this is the first case report of bullous AE in the Korean dermatologic literature.


Asunto(s)
Humanos , Masculino , Absorción , Acrodermatitis , Alopecia , Vesícula , Nalgas , Citocromo P-450 CYP1A1 , Dermis , Diarrea , Extremidades , Dedos , Queratinocitos , Linfocitos , Uñas , Neutrófilos , Pancreatitis , Nutrición Parenteral Total , Leucemia-Linfoma Linfoblástico de Células Precursoras , Estomatitis , Dedos del Pie , Zinc
10.
Korean Journal of Dermatology ; : 529-532, 2011.
Artículo en Coreano | WPRIM | ID: wpr-148863

RESUMEN

Zinc deficiency in breast-fed infants is an important disorder. Unlike acrodermatitis enteropathica, it is transient and stops when nursing ends. We report on a case of a breast-fed, full-term male infant who presented with erythematous fine scaly patches and plaques involving the flexural area and trunk at 4 month of age. Clinical impressions of intertrigo, fungal infection or inverse psoriasis were considered. These lesions did not respond to treatment with topical antifungal agents and corticosteroids. The results of laboratory investigations revealed a lowered zinc level in the infant's serum (48.4microg/dl: normal 70~121microg/dl) and in the mother's milk (16microg/dl: normal 48~75microg/dl) and a normal level of zinc in the mother's serum. After the patient's mother was given zinc supplements, the skin lesions were dramatically improved and his serum zinc level became normal. Early recognition of the disorder and introduction of zinc supplementation rapidly reversed the transient zinc deficiency.


Asunto(s)
Humanos , Lactante , Masculino , Acrodermatitis , Corticoesteroides , Antifúngicos , Intertrigo , Leche , Madres , Psoriasis , Piel , Zinc
11.
Journal of the Korean Society of Neonatology ; : 383-386, 2011.
Artículo en Coreano | WPRIM | ID: wpr-59451

RESUMEN

Acrodermatitis enteropathica due to zinc deficiency is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Here we report a case of transient acrodermatitis enteropathica in a 2-month-old preterm infant, whose skin is erythematous, scaly, eczematous, with an eruption starting from the periorificial area. While her serum zinc level was normal at 118.2 ug/dL, the serum alkaline phosphatase level was low at 48 IU/L and the serum alkaline phosphatase level get increased concordant with clinical improvement after zinc supplementation. Hence, this case shows that the diagnosis of zinc deficiency can be aided with a low level of serum alkaline phoaphatase although serum zinc level is normal.


Asunto(s)
Humanos , Lactante , Recién Nacido , Acrodermatitis , Fosfatasa Alcalina , Alopecia , Dermatitis , Diarrea , Recien Nacido Prematuro , Piel , Zinc
12.
Indian J Pediatr ; 2010 July; 77(7): 805-806
Artículo en Inglés | IMSEAR | ID: sea-142636

RESUMEN

A 2-week-old infant born at 36-week gestation developed diarrhea and metabolic acidosis when he was put on formula feeding. He was treated for sepsis and was screened for metabolic diseases. Blood and cerebrospinal fluid cultures were clear. The diarrhea and metabolic acidosis settled but recurred when formula feeding was resumed. He developed a florid erythematous rash involving the palms, feet, perioral and perineal regions. Zinc deficiency was confirmed and zinc replacement resulted in prompt resolution of the skin rash. The patient was put on Pepti-Junior and remained well. This case illustrates that zinc deficiency must be sought and treated in an infant with a typical rash involving the palms, feet and body orifices.


Asunto(s)
Acidosis/etiología , Acrodermatitis/etiología , Enfermedades Carenciales/complicaciones , Enfermedades Carenciales/diagnóstico , Diagnóstico Diferencial , Diarrea Infantil/etiología , Humanos , Recién Nacido , Intolerancia a la Lactosa/complicaciones , Intolerancia a la Lactosa/diagnóstico , Masculino , Hipersensibilidad a la Leche/diagnóstico , Zinc/deficiencia
13.
GED gastroenterol. endosc. dig ; 29(1): 32-35, jan.-mar. 2010. ilus
Artículo en Inglés | LILACS | ID: lil-571929

RESUMEN

Acrodermatitis enteropathica (AE) is a rare, recessively inherited disorder of zinc metabolism, which usually presents in infancy, at the time of weaning, with the triad alopecia, diarrhea and dermatitis with lesions of periorificial and acral distribution. We report a full-term 2-year-old boy with typical skin leions, decreased plasma zinc and alkaline phosphatase levels, that improved with zinc sulfate supplementation. He presented relapse of the symptoms and lesions when the medication was interrupted, in several occasions, suggesting treatment for long-life. A bried historial report and current genetics concepts are discussed. Currently, gene 8p24.3, SLC39A4, in the apical membrane of the enterocytes, was implicated in AE. In Brazil there are few reports of AE.


Acrodermatitis enteropathica (AE) é um distúrbio recessivo raro do metabolismo do zinco que habitualmente se apresenta à época do desmame. A tríade característica é alopecia, diarreia e dermatite com lesões periorificiais e de distribuição acral. Relata-se o caso de um menino de 2 anos de idade, nascido a termo, alimentado com leite de vaca, com lesões de pele típicas de AE, tendo níveis plasmáticos reduzidos de zinco e de fosfatase alcalina. A suplementação de zinco acarretou melhora do quadro, mas sintomas e lesões reapareceram quando a medicação foi interrompida, em diferentes épocas, sugerindo que deva ser continuada indefinidamente. Faz-se breve revisão histórica e dos conceitos genéticos atuais. Gene na região 8p24.3, SLC39A4, expresso na membrana apical dos enterócitos, está relacionado do com a AE. No Brasil há poucos relatos desta afecção.


Asunto(s)
Humanos , Masculino , Preescolar , Zinc , Acrodermatitis , Dermatitis , Diarrea Infantil , Alopecia
14.
Journal of Korean Medical Science ; : 1818-1820, 2010.
Artículo en Inglés | WPRIM | ID: wpr-61706

RESUMEN

Acrodermatitis enteropathica (AE) is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. AE is known to be caused by mutations of the SLC39A4 gene on the chromosome band 8q24.3, encoding the zinc transporter in human. An 8-month-old Korean boy presented with eczematous changes on the inguinal area and knees and was diagnosed with AE. Blood tests revealed a markedly decreased level of plasma zinc, and his symptoms improved on oral zinc replacement. To confirm the diagnosis of AE from congenital zinc deficiency, direct sequencing analysis of SLC39A4 was performed and revealed that he was compound heterozygous for a known missense mutation (Arg95Cys) and a novel splicing mutation in the donor site of intron 7 (c.1287+2T>C). Family study showed that his parents were heterozygous carriers of the mutations. To the best of our knowledge, this is the first report of genetically confirmed AE in Korea.


Asunto(s)
Humanos , Lactante , Masculino , Acrodermatitis/congénito , Empalme Alternativo , Proteínas de Transporte de Catión/genética , Cromosomas Humanos Par 8 , Heterocigoto , Mutación Missense , Análisis de Secuencia de ADN , Zinc/sangre
15.
Korean Journal of Dermatology ; : 1314-1317, 2009.
Artículo en Coreano | WPRIM | ID: wpr-213807

RESUMEN

A male full-term infant who had been breast-fed since birth developed an erythematous eroded eruption involving the anogenital area at 1 month of age, and this lesion did not respond to treatment with topical corticosteroids and antimicrobials. He was first seen at our office at 9 months of age. At that time, he had continuous diarrhea, but he had no alopecia, nail dystrophy or acral skin lesions. His serum zinc level was normal and his mother's breast milk zinc level was also normal, but it was not enough for his zinc metabolism. His clinical symptoms and skin lesions were dramatically improved with administering zinc.


Asunto(s)
Humanos , Lactante , Masculino , Acrodermatitis , Corticoesteroides , Alopecia , Citocromo P-450 CYP1A1 , Diarrea , Leche Humana , Uñas , Parto , Piel , Zinc
16.
Korean Journal of Pediatrics ; : 209-212, 2007.
Artículo en Inglés | WPRIM | ID: wpr-71845

RESUMEN

Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder of early infancy, and is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Serum zinc levels are usually low in untreated patients and the oral administration of zinc sulfate can clear skin lesions and other symptoms. Although premature and cow`s milk-fed infants are at particular risk of developing AE, there have been a few reports about AE in term and breast-fed infants. We report a case of transient AE in a 4-month-old breast-fed infant. This patient suffered from diarrhea and dermatitis for more than a month. Her skin lesions were erythematous, scaly, crusted, psoriasiform, eczematous, with an eruption at the chin, and a periorificial disposition with involvement of the flexural areas of lower extremities. Her serum zinc level was almost normal at 129 microgram/dL (reference range: 60-121 microgram/dL), but the zinc level in her hair was low: 8 mg percent (reference range: 10-21 mg percent). Skin biopsy findings were consistent with AE. Seven days after zinc supplementation, the skin lesions and diarrhea improved. The authors recommend that a clinical trial of zinc supplementation be considered in cases where there are suspicious of AE, even when the serum zinc level is normal.


Asunto(s)
Humanos , Lactante , Acrodermatitis , Administración Oral , Alopecia , Biopsia , Mentón , Dermatitis , Diarrea , Cabello , Extremidad Inferior , Piel , Sulfato de Zinc , Zinc
17.
Korean Journal of Dermatology ; : 203-205, 2007.
Artículo en Coreano | WPRIM | ID: wpr-24307

RESUMEN

A 5 month-old, breast-fed, full-term female infant presented with an acral and periorificial dermatitis. Her serum zinc level was low. Her mother's serum zinc level was normal and breast milk zinc was slightly reduced. The infant responded rapidly to zinc therapy. After interruption of zinc therapy, she showed slightly reduced zinc levels, but no relapse, while receiving powdered milk and other baby foods. To maintain zinc serum level, zinc was readministered for 4 weeks. Follow-up zinc levels were normal after discontinuation of the zinc readministration. A diagnosis of transient acrodermatitis enteropathica was made based on the above findings.


Asunto(s)
Femenino , Humanos , Lactante , Acrodermatitis , Dermatitis , Diagnóstico , Estudios de Seguimiento , Leche , Leche Humana , Recurrencia , Zinc
18.
Journal of Korean Medical Science ; : 908-911, 2005.
Artículo en Inglés | WPRIM | ID: wpr-152997

RESUMEN

We present here a case of acrodermatitis enteropathica-like eruption associated with essential free fatty acid and protein deficiencies as well as borderline zinc deficiency that occurred after Whipple's operation in a 31-yr-old woman. Her eruptions were improved not by zinc supplements alone, but her condition was improved by total parenteral nutrition including amino acids, albumin, lipid and zinc. Although we could not exactly decide which of the nutrients contributed the most to her manifestations, we inferred that all three elements in concert caused her dermatoses. This case shows that even though the patient's skin manifestations and laboratory results are suggestive of acrodermatitis enteropathica, the physicians should keep in mind the possibility that this disease can be associated with other nutritional deficiencies such as free fatty acid or protein deficiency.


Asunto(s)
Adulto , Femenino , Humanos , Acrodermatitis/diagnóstico , Ácidos Grasos Esenciales/deficiencia , Desnutrición/diagnóstico , Pancreatectomía/efectos adversos , Enteropatías Perdedoras de Proteínas/diagnóstico , Enfermedades de la Piel/diagnóstico , Zinc/deficiencia
19.
Korean Journal of Dermatology ; : 786-789, 2003.
Artículo en Coreano | WPRIM | ID: wpr-50964

RESUMEN

Acrodermatitis enteropathica is a rare autosomal recessive disorder affecting early infancy. This syndrome is characterized by acral and periorificial dermatitis, alopecia and intractable diarrhea. Whereas hereditary acrodermatitis enteropathica is caused by mutation affecting intestinal zinc absorption, transient acrodermatitis enteropathica is caused by inadequate supply or malabsorption of zinc in the premature infant. We report a case of transient acrodermatitis enteropathica in a 5-month-old, breast-fed premature infant.


Asunto(s)
Humanos , Lactante , Recién Nacido , Absorción , Acrodermatitis , Alopecia , Dermatitis , Diarrea , Recien Nacido Prematuro , Zinc
20.
Journal of the Korean Pediatric Society ; : 469-474, 2001.
Artículo en Coreano | WPRIM | ID: wpr-28406

RESUMEN

Maple syrup urine disease(MSUD) is an autosomal recessive disorder involving the metabolism of the branched-chain amino acids(BCAA) such as leucine, isoleucine and valine. The disorder is due to a defect in branched-chain alpha-ketoacid dehydrogenase(BCKAD) and the classic form causes rapid progressive and overwhelming illness beginning in the first weeks of life, present with poor feeding, lethargy, change in muscle tone, acidosis, seizures and coma. The goal of therapy in acutely ill patients with MSUD is an immediate reduction in the plasma levels of the BCAAs and branched-chain ketoacids. In this report, we describe an infant with MSUD who was treated by dietary therapy alone. During the therapy, acrodermatitis enteropathica-like syndrome developed with low plasma isoleucine concentration while she was receiving a formula deficient in BCAAs.


Asunto(s)
Humanos , Lactante , Acer , Acidosis , Acrodermatitis , Aminoácidos de Cadena Ramificada , Coma , Dietoterapia , Dieta , Isoleucina , Letargia , Leucina , Enfermedad de la Orina de Jarabe de Arce , Metabolismo , Plasma , Convulsiones , Valina
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