Meningitis por Salmonella tras toxiinfección alimentaria en lactante alimentado con lactancia artificial / Meningitis due to Salmonella after food poisoning in an infant fed with formula milk

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9 Years after the first laparoscopic adjusted gastric banding (LAGB) in adolescents: the Portuguese experience.

INTRODUCTION: Children all over the world are increasingly becoming overweight and obese. The experience with adults has demonstrated that surgery is the only effective way of achieving sustained weight loss in obese patients. Simultaneously, it has been proposed that bariatric surgery in the adolescent period would be a more effective treatment for childhood-onset extreme obesity rather than delaying surgery for extremely obese youth until adulthood. Nevertheless, the optimal surgical option for both adults and adolescents has yet to be established. The aim of our study was to evaluate the results of 9 years of laparoscopic adjusted gastric banding (LAGB) performed in our hospital for obesity-associated comorbidities (OAC), weight loss and surgical morbidity. METHODS: A prospective study of all patients who underwent LAGB at our hospital between July 2001 and May 2010 was carried out. Patient selection was by a multidisciplinary team and based on the "IPEG Guideline for the Surgical Treatment of Extremely Obese Adolescents". Data were reviewed retrospectively. RESULTS: 14 patients underwent LAGB (10♀; 4♂; average age: 16 years). Preoperative BMI ranged between 41.4 and 54.6 kg/m (2). Prior to surgery, 8 different OACs were identified. 6 months later, 4 types of OAC had resolved completely and the other 4 had improved, and the improvement was maintained over time. The average excess weight (EW) loss increased from 25.7 to 48.2% at 6 and 36 months of follow-up, respectively (10 patients). At 60 and 106 months of follow-up it was 41.5% (5 patients) and 31.8% (1 patient), respectively. There were no complications arising from the surgery itself. Long-term complications were few and easily managed. CONCLUSIONS: LAGB seems to be a good option to treat obese adolescents, as it is a minimally invasive procedure that does not radically change the patient's anatomy and is associated with minimal morbidity. It leads to a sustained improvement/resolution of OAC, and although weight loss is not continuous, it is maintained over time.

La investigación biomédica, el mercado, los clínicos, la seguridad y la responsabilidad social corporativa posfase III: retener la confianza / Biomedical research, the market, clinicians, safety and corporate social responsibility post-phase III: maintaining confidence

Introducción: Los ensayos controlados aleatorios son el gold standar occidental respecto a la toma de decisiones, tanto para los clínicos como para los gestores o responsables de políticas comunitarias. En este poderoso despliegue de esfuerzo investigador existen grados variables de conflictos de intereses y los clínicos, que no somos ajenos a eso, estamos entrando en un mar de dudas acerca de la seguridad, dimensión que emerge como un elemento diacrítico, irrenunciable. El objeto del estudio fue toda evaluación crítica y la selección sobre editoriales, ensayos clínicos o metaanálisis publicados en soporte físico o vía web, acreditados internacionalmente, que resultaran más relevantes y que abordaran como objetivo primario la seguridad de los pacientes. Pacientes y métodos: Investigación evaluativa que se llevó a cabo en el Complejo Hospitalario Torrecárdenas, perteneciente al Servicio Andaluz de Salud. Sistema Sanitario Público de Andalucía, en el contexto de una unidad formativa acreditada (Mejora_F) en 2008, denominada «transferencia del conocimiento biomédico desde la bibliografía». Resultados: Los artículos analizados fueron 170 (el 75% en inglés, con predominio de New England Journal of Medicine [>50%], seguida de Journal Of the American Medical Association, The Lancet, British Medical Journal, Journal of American Geriatrics, Medicina Clínica, Revista Clínica Española e Infectología). De los 170 artículos elegibles, seleccionamos 5 tópicos clave por su impacto como los más representativos por su frecuencia de citación. Representaron, a nuestro juicio, «una bandera roja» de seguridad los siguientes: betaestimulantes de acción larga y aumento de mortalidad en asma; neurolépticos en ancianos y prolongación del intervalo QT asociado a muerte súbita; tiazolidindiona en diabetes de tipo ii y efectos negativos cardiovasculares; potenciación de estatinas-ezetimiba y discutible asociación con mayor mortalidad por cáncer, y tratamiento intensivo en diabetes y probable aumento de mortalidad. Conclusiones: Lo que realmente importa en biomedicina es qué efectos provoca una estrategia dada en los pacientes reales, no en los puntos intermedios. Los clínicos no deberíamos apoyar resultados parciales de diseños basados en datos intermedios -indudablemente menos costosos y más rápidos- sin conocer adecuadamente la seguridad de la estrategia erigida para alcanzarlos. Sólo así nos constituimos en verdaderos garantes de la seguridad, sólo así, y en ausencia de conflictos de intereses, podremos mantener la confianza dada (AU)

Introduction: Randomised controlled trials (RCTs) are the gold standard in the western world for decision making, as much for the clinicians as for the agencies or managers for community policies. In this powerful deployment of investigative effort there are variable degrees of conflict of interests, and the clinicians, not foreign to this, are entering a sea of doubts on safety, a dimension that emerges like a diacritical, inalienable element. The aim of the study was to select and ctically evaluate editorials, clinical trials and/or meta-analyses published on physical support, or the more important internationally credited websites, which has patient safety as their primary objective. Patients and Methods: Evaluative study performed in the Torrecardenas Hospital, of the Andalucian Public Health Service (SSPA), in the context of an accredited training unit (Mejora_F), so called "transference of the biomedical knowledge from the bibliography" from 2008 to 2009. Results: Analyzed articles: 170 (75 % in English, with predominance of N Eng J Med >50 %; followed by Journal Of the American Medical Association, The Lancet, British Medical Journal, Journal of American Geriatrics; Med Clin, Rev Clin Esp.; and Infectología). From 170 eligible articles we selected 5 key topoi due to their impact as the most representative owing to their citation frequency. They represented, in our judgment, "a red flag" of safety: long-acting beta-stimulators or LABS and increase in mortality in asthma; neuroleptics in the elderly and extension of the QT interval associated with sudden death; thiazolidinediones in type II diabetes and negative cardiovascular effects; promotion of statins-ezetimibe and the debatable association with major mortality for cancer, and intensive treatment in diabetes and probable increase in mortality. Conclusions: What really maters in biomedicine is that it leads to a given strategy in real patients, not the intermediate points. Clinicians should not support partial results of designs based on intermediate information -undoubtedly less costly and more rapid- without adequately knowing the safety of the strategy built to reach them. Only in this way we can be real guarantors of safety, and only in this way, and in absence of conflicts of interests we will be able to support the given confidence (AU)

[Biomedical research, the market, clinicians, safety and corporate social responsibility post-phase III: maintaining confidence]. / La investigación biomédica, el mercado, los clínicos, la seguridad y la responsabilidad social corporativa posfase III: retener la confianza.

INTRODUCTION: Randomised controlled trials (RCTs) are the gold standard in the western world for decision making, as much for the clinicians as for the agencies or managers for community policies. In this powerful deployment of investigative effort there are variable degrees of conflict of interests, and the clinicians, not foreign to this, are entering a sea of doubts on safety, a dimension that emerges like a diacritical, inalienable element. The aim of the study was to select and ctically evaluate editorials, clinical trials and/or meta-analyses published on physical support, or the more important internationally credited websites, which has patient safety as their primary objective. PATIENTS AND METHODS: Evaluative study performed in the Torrecardenas Hospital, of the Andalucian Public Health Service (SSPA), in the context of an accredited training unit (Mejora_F), so called "transference of the biomedical knowledge from the bibliography" from 2008 to 2009. RESULTS: Analyzed articles: 170 (75 % in English, with predominance of N Eng J Med >50 %; followed by Journal Of the American Medical Association, The Lancet, British Medical Journal, Journal of American Geriatrics; Med Clin, Rev Clin Esp.; and Infectología). From 170 eligible articles we selected 5 key topoi due to their impact as the most representative owing to their citation frequency. They represented, in our judgment, "a red flag" of safety: long-acting beta-stimulators or LABS and increase in mortality in asthma; neuroleptics in the elderly and extension of the QT interval associated with sudden death; thiazolidinediones in type II diabetes and negative cardiovascular effects; promotion of statins-ezetimibe and the debatable association with major mortality for cancer, and intensive treatment in diabetes and probable increase in mortality. CONCLUSIONS: What really maters in biomedicine is that it leads to a given strategy in real patients, not the intermediate points. Clinicians should not support partial results of designs based on intermediate information - undoubtedly less costly and more rapid - without adequately knowing the safety of the strategy built to reach them. Only in this way we can be real guarantors of safety, and only in this way, and in absence of conflicts of interests we will be able to support the given confidence.

Documento de Consenso sobre el posible empleo de Ácido Conjugado Linoleico (CLA), como complemento alimenticio, en la infancia y la adolescencia / Consensus Document of possible use of Conjugated Linoleic Acid (CLA), as dietary supplement in childhood and adolescence

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Adipose tissue and liver lipid metabolism in obese children: role of the body mass index and the presence of acanthosis nigricans.

AIMS: The aims of our study were to determine if insulin resistance is associated with increased plasma levels of non-esterified fatty acids (NEFA), glycerol, 3-hydroxybutyrate and triglycerides in obese children. We also studied whether the presence of acanthosis nigricans (AN) led to further alterations in the above parameters. METHODS: A total of 101 children were studied on their first visit to the paediatric endocrine clinic. Seventy-four were obese, 30 of them with AN. The remaining 27 were non-obese healthy children (control group). NEFAs, glycerol, triglycerides, 3-hydroxybutyrate, insulin, leptin, adiponectin and glucose were determined in blood samples obtained after overnight fasting. The insulin resistance index (IRI) was calculated following the homeostasis model assessment (HOMA). Data from the three groups were compared using appropriate statistical tests. RESULTS: No differences in age, sex ratio and pubertal stage were observed among the three groups. The group of children with the highest body mass index (BMI) showed higher plasma levels of insulin and leptin, higher IRI and lower plasma levels of adiponectin. As insulin and IRI increased, NEFA and 3-hydroxybutyrate decreased and triglycerides increased. When obese children were categorized by BMI, the presence of AN further exacerbated these differences. CONCLUSIONS: In obese children, insulin resistance is associated with plasma lipid alterations suggestive of both decreased adipose tissue lipolysis and hepatic beta-oxidation and increased hepatic synthesis of triglycerides. Such a metabolic condition may facilitate fat storage and hinder weight loss.

Clastogenic and cytotoxic effects of lipid peroxidation products generated in culinary oils submitted to thermal stress.

In rats, the administration of oil submitted to high temperatures is teratogenic. To understand the mechanisms involved, the clastogenic and cytotoxic effects were studied respectively in lymphocytes, hepatocytes (HepG2) and in human umbilical vein endothelial cells (HUVEC) cultured with a water extract obtained from heated oil (HO). In lymphocytes incubated with HO containing 0.075 or 0.15 microM of thiobarbituric reacting substances, the rate of chromosomal breakage was 18.4% and 23.1%, compared to 8.7% and 6.6%, or 8.1% and 9.2%, respectively in lymphocytes incubated with the same volume of a water extract from non-heated oil (NHO) or distilled water (DW). In HepG2 or HUVEC cells, the cytotoxic properties of the HO were dose dependent, the cytotoxicity beginning at concentrations as low as 0.25 microM. In contrast, the same volume of NHO or DW was non-toxic for these cells. The results obtained show that a water extract obtained from heated oil is clastogenic and, in higher doses, cytotoxic.

[Virus and systemic vasculitis]. / Virus y vasculitis sistémicas.

The participation of genetic and environmental factors has always been invoked in the pathogenesis of the autoimmune systemic diseases, including the primary vasculitides. Among the environmental factors, infections, fundamentally those having a viral nature, have always been focused on, especially after the discovery of the close existing relationship between the polyarteritis nodosa and the hepatitis B virus, on the one hand, and mixed cryoglobulinemia and the hepatitis C virus, on the other. The present review summarizes data from the most recent literature related to associations between virus infections and primary vasculitides, following the Chapel-Hill vasculitis classification.

Virus y vasculitis sistémicas / Virus and systemic vasculitis

En la patogenia de las enfermedades autoinmunes sistémicas, incluyendo las vasculitis, siempre se ha invocado la participación de factores genéticos y factores ambientales. Entre los ambientales, las infecciones, fundamentalmente de naturaleza vírica, han sido siempre un foco de atención, máxime tras el descubrimiento de la estrecha relación existente entre la poliarteritis nudosa y el virus de la hepatitis B, por una parte, y la crioglobulinemia mixta esencial y el virus de la hepatitis C, por otra. La presente revisión pretende actualizar los conocimientos recogidos en la literatura más reciente sobre el binomio virus y vasculitis sistémicas, siguiendo la clasificación de vasculitis de Chapel-Hill

The participation of genetic and environmental factors has always been invoked in the pathogenesis of the autoimmune systemic diseases, including the primary vasculitides. Among the environmental factors, infections, fundamentally those having a viral nature, have always been focused on, especially after the discovery of the close existing relationship between the polyarteritis nodosa and the hepatitis B virus, on the one hand, and mixed cryoglobulinemia and the hepatitis C virus, on the other. The present review summarizes data from the most recent literature related to associations between virus infections and primary vasculitides, following the Chapel-Hill vasculitis classification

Long-term reversal of hypocholesterolaemia in patients with chronic hepatitis C is related to sustained viral response and viral genotype.

BACKGROUND: Genotype-3 of hepatitis C virus (HCV) has been associated with serum lipid changes (reversible with sustained viral response) and liver steatosis. AIM: To characterize the relationships among hepatic steatosis, cholesterol and sustained viral response in these patients. METHODS: Patients (n = 215) with chronic hepatitis C (157 with genotype-1 of HCV) had age, body mass index, gender, alcohol intake, glycaemia, serum lipids, transaminases, grade and stage (METAVIR and Scheuer), degree of liver steatosis, sustained viral response, insulinaemia, leptinaemia, beta-hydroxybutyrate and glycerol measured, and were compared with 32 hepatitis B virus (HBV)-infected subjects. RESULTS: Genotype-3 of HCV patients had age-adjusted hypocholesterolaemia and more frequent hepatic steatosis (P < 0.001). Steatosis was inversely correlated with serum cholesterol (P < 0.01) and directly with viral load (P < 0.03). In patients with genotype-3 of HCV and sustained viral response, serum cholesterol increased from 138 (95% CI: 120-151) to 180 mg/dL (95% CI: 171-199) 12 months after treatment conclusion (P < 0.0001). By contrast, cholesterol values were unchanged in genotype-3 of HCV non-responders and in patients with genotype-1 of HCV regardless of response. Rising cholesterol in sustained viral response did not parallel the changes in beta-hydroxybutyrate. CONCLUSIONS: Besides causing hepatic steatosis, genotype-3 specifically decreases serum cholesterol. This interference with the metabolic lipid pathway is related to viral load, is reversed with sustained viral response, and seems unrelated to mitochondrial dysfunction.

[Minimal invasive surgical of intersex abnormalities: our experience]. / Cirugía mínimamente invasiva en el intersexo: nuestra experiencia.

The term intersex makes reference to either newborn infants who show ambiguous external genitals or those who, having a normal phenotype, show growth alterations in the puberty. The etiologic diagnostic is oriented by the physical examination, biochemical studies, radiologic, surgical techniques and histological discoveries; that enables us to decide one more accurate sex assignment; the laparoscopy plays an important role, for the direct visualisation of the intra-abdominal gonads, internal genital identification, gonadal biopsy, as well as therapeutic interventions as gonadectomy, orchidopexy and resection of Müllerian remants, replacing totally the laparotomy. The purpose of our job was evaluate the role of the minimal invasive surgical as diagnostic and treatment of the intersex, in nine intersex children treated in our hospital over the last three years.

[Reconstruction of velopharyngeal sphincter in secondary Cleft Palate: surgical alternative]. / Reconstrucción del esfínter velofaríngeo en la fisura del paladar secundario: alternativa quirúrgica.

There are numerous surgical procedures for the repair of Cleft Palate (CP). Since 1998, in children with CP we use a modified Wardill-Kilner technique, with a large section of the nasal mucous layer at the level of the muscular insertion on the hard palate and lateral nasopharingeal wall, obtaining a push-back and reorientation of the muscular fibres without dissection, diminishing this way the risks of haemorrhages and fibrosis, simplifying the intervention. It allows a lower operative time and a short internment. The aim of our study was to evaluate the results of this operative procedure specially in the development of the speech in 73 children operated on from 1998 until 2000 in our hospital. We verify a competence of the velopharingeal sphincter with ideal results in speech in 88,8% of the cases.

Cirugía mínimamente invasiva en el intersexo: nuestra experiencia / Minimal invasive surgical of intersex abnormalities: our experience

El término intersexo hace referencia bien a aquellos recién nacidos que al nacer presentan los genitales externos ambiguos o bien aquellos que, teniendo un fenotipo normal, presentan alteraciones del desarrollo en la pubertad o adolescencia. Es a través de la exploración clínica, bioquímica, radiología, técnicas quirúrgicas y hallazgos anatomopatológicos, que nos permiten orientar el diagnóstico etiológico y decidir así una asignación del sexo más precisa. La laparoscopia juega un importante papel, no sólo para la visualización directa de las gónadas intraabdominales, identificación de los genitales internos o biopsia gonadal, sino también como medida terapéutica para la realización de gonadectomía, orquidopexia o exéresis de restos müllerianos, desplazando totalmente a la laparotomía. Es en esta perspectiva, que el objetivo de nuestro trabajo fue evaluar el papel de la cirugía mínimamente invasiva como medio diagnóstico y terapéutico del intersexo, en 9 niños tratados en nuestro Centro durante los últimos 3 años (AU)

The term intersex makes reference to either newborn infants who show ambiguous external genitals or those who, having a normal phenotype, show growth alterations in the puberty. The etiologic diagnostic is oriented by the physical examination, biochemical studies, radiologic, surgical techniques and histological discoveries; that enables us to decide one more accurate sex assignment; the laparoscopy plays an important role, for the direct visualisation of the intra-abdominal gonads, internal genital identification, gonadal biopsy, as well as therapeutic interventions as gonadectomy, orchidopexy and resection of müllerian remants, replacing totally the laparotomy. The purpose of our job was evaluate the role of the minimal invasive surgical as diagnostic and treatment of the intersex, in nine intersex children treated in our hospital over the last three years (AU)

Reconstrucción del esfínter velofaríngeo en la fisura del paladar secundario: alternativa quirúrgica / Reconstruction of velopharyngeal sphincter in secondary cleft palate: surgical alternative

Existen numerosas técnicas quirúrgicas para la reparación de las fisuras del paladar secundario (FPS). Desde 1998, en niños con FPS usamos la técnica de Wardill-Kilner modificada, realizando sección de la mucosa nasal a nivel de la inserción de los músculos del paladar en las apófisis palatinas, consiguiendo retroceder y reorientar las fibras musculares sin disección de las mismas, disminuyendo así los riesgos de hemorragia y fibrosis, simplificando la intervención. Permite un tiempo operatorio y de internado corto. El objetivo de nuestro estudio fue evaluar los resultados de esta técnica principalmente en el desenvolvimiento del habla en 73 niños operados desde 1998 hasta 2000 en nuestro hospital. Verificamos una competencia del esfínter velofaríngeo, con óptimos resultados en el habla en 88,8% de los casos (AU)

There are numerous surgical procedures for the repair of Cleft Palate (CP). Since 1998, in children with CP we use a modified Wardill-Kilner technique, with a large section of the nasal mucous layer at the level of the muscular insertion on the hard palate and lateral nasopharingeal wall, obtaining a push-back and reorientation of the muscular fibres without dissection, diminishing this way the risks of haemorrhages and fibrosis, simplifying the intervention. It allows a lower operative time and a short internment. The aim of our study was to evaluate the results of this operative procedure specially in the development of the speech in 73 children operated on from 1998 until 2000 in our hospital. We verify a competence of the velopharingeal sphincter with ideal results in speech in 88,8% of the cases (AU)

[Nonpalpable testes: experience from the Hospital Central Especializado de Crianças Maria Pia]. / Testiculos no palpables: experiencia del Hospital Central Especializado de Crianças Maria Pia.

The incidence of cryptorchidism in children at age of 1 year is 1%, and 20% of these cases represent nonpalpable testes. The aim was to evaluate the use of minimal invasive surgery for the diagnosis and therapy of these patients. The authors made a prospective, analytical and longitudinal study from January 2001 through December 2004 in 89 consecutive boys undergoing laparoscopy for 98 impalpable testes. The following details were analyzed: examination under general anesthesia, ultrasound examination, age at surgery, findings at laparoscopy, operative procedure, results of the histology and follow up.

Neuroprotective effect of L-arginine in a newborn rat model of acute severe asphyxia.

The left common carotid artery was ligated in anaesthetized 7-day-old Wistar rats (P7), prior to asphyxia by inhaling 100% nitrogen for 9 min. Pups recovered from asphyxia received i.p. saline (n = 16), or L-Arg 300 mg/kg (n = 14). Pups undergoing sham operation remained as controls (n = 12). At day 14, the amount of surviving or degenerating neurons was quantified under optical microscopy by Nissl technique or by Fluoro-Jade B (FJB) in CA1 area of hippocampus and in parietal cortex. In these areas, asphyxia reduced the neuronal density by 23.6 and 30%, and increased the proportion of degenerating neurons two and four times, respectively. L-Arg administration to asphyxiated pups reduced the neuronal loss and the proportion of degenerating neurons by 50% (p < 0.05). We conclude that L-Arg administration after acute severe asphyxia in newborn rats is neuroprotective, reducing early and delayed neuronal loss.

Effects of aldehydes on CD36 expression.

INTRODUCTION: During the oil frying process lipid peroxidation compounds are formed. These products can modulate gene expression and alter cellular behaviour. The cellular uptake of oxidized LDL, a key step in the development of atherosclerosis, is mediated by the CD36 scavenger receptor, whose expression is down-regulated by alpha-tocopherol. OBJECTIVE: To determine the effects of water-soluble aldehydes, obtained from thermally oxidized sunflower oil on the expression of CD36 scavenger receptor in human monocytes (THP-1 cells). We also wanted to study the effects of alpha-tocopherol on CD36 expression in the presence of water-soluble aldehydes. MATERIALS AND METHODS: Sunflower oil was heated in a frying pan, at 180--200 degrees C for 40 min, water-soluble aldehydes were isolated, and the content of thiobarbituric acid reacting substances (TBARS) was determined. THP-1 monocytes were cultured in RPMI medium during 24 h and incubated with increasing concentrations of the water-soluble aldehydes (ranging from 0.05 to 1 microM) and with or without 50 microM of alpha-tocopherol. In parallel, THP-1 cells were cultured with the same volume of an extract obtained from non-oxidized oil or distilled water. The CD36 expression at the cell surface was studied with fluorescence-activated cell sorting (FACS). RESULTS: Monocytes incubated in a medium containing water-soluble aldehydes, showed a dose dependent increase in the expression of the CD36 protein on the cell surface, compared to with the control groups. When the cells were treated simultaneously with 50 microM of alpha-tocopherol a significant reduction in the expression of the CD36 protein was observed. CONCLUSION: Water-soluble aldehydes, extracted from thermally oxidized culinary oil, increase the expression of CD36. This effect is partially decreased by the presence of alpha-tocopherol.

Resolución de las alteraciones metabólicas y endocrinológicas asociadas a la lipodistrofia congénita tras el tratamiento con leptina / Resolution of metabolic and endocrinological alterations associated with congenital lipodystrophy after leptin treatment

La lipodistrofia congénita es una enfermedad poco frecuente que se caracteriza por la ausencia de tejido adiposo subcutáneo y una elevada resistencia a la insulina. Se presenta el caso de una paciente con lipodistrofia congénita y déficit de leptina (hormona producida por el tejido adiposo, que interviene, entre otras funciones, en la regulación de la ingesta y el metabolismo intermediario). Entre las alteraciones físicas destacan rasgos acromegálicos, acantosis nigricans y hepatomegalia. Las alteraciones metabólicas y endocrinológicas asociadas fueron hiperglucemia, a pesar del tratamiento con elevadas dosis de insulina, hipertrigliceridemia, bajos valores de colesterol ligado a lipoproteínas de alta densidad, amenorrea y esteatohepatitis. Tras la inclusión de la paciente en un ensayo clínico donde se administró leptina se produjo la completa remisión de las alteraciones metabólicas y endocrinológicas. En el presente artículo se comentan los posibles mecanismos fisiopatológicos. Además se analiza qué hallazgos podrían deberse exclusivamente al déficit de leptina y cuáles al hiperinsulinismo asociado al cuadro, ya que ambas alteraciones están estrechamente ligadas (AU)

Congenital lipodystrophy is an uncommon entity characterized by the absence of subcutaneous adipose tissue and elevated insulin resistance. We report the case of a woman with congenital lipodystrophy and leptin deficiency (a hormone produced by adipose tissue, which, among other functions, plays a role in the regulation of food intake and intermediate metabolism). Notable among the patient's physical alterations were acromegalic features, acanthosis nigricans, and enlarged liver. Associated metabolic and endocrinological features were hyperglycemia, despite treatment with high-dose insulin, hypertriglyceridemia, low high-density lipoprotein cholesterol, amenorrhea, and steatohepatitis. The patient was enrolled in a clinical trial that administered leptin and showed complete remission of the metabolic and endocrinological alterations. The present report discusses the possible physiopathological mechanisms involved. We also discuss which findings could be exclusively due to leptin deficiency and which findings were probably due to the hyperinsulinism associated with congenital lipodystrophy, since both alterations are closely linked (AU)