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1.
Arch Pediatr ; 30(4): 212-218, 2023 May.
Artículo en Inglés | MEDLINE | ID: mdl-37061360

RESUMEN

BACKGROUND: Little is known about blood lymphocyte subpopulations in children with common (CO) or syndromic (SO) obesity. We aimed to describe the blood lymphocyte profiles of obese children and to search for associations with clinical phenotypes. METHODS: Main blood lymphocyte subpopulations were analyzed in 159 children with CO and 34 with SO in a retrospective cohort. Phenotypes included obesity history, body mass index (BMI) Z score, percentage fat mass, and inflammatory parameters. Correlations were performed between phenotypes and circulating lymphocyte profiles. RESULTS: Children with SO had a higher BMI Z score (5.5 ± 1.7 SD) than children with CO (4.7 ± 0.9 SD; p = 0.01). Significant differences were found for lymphocyte counts, including a higher percentage of CD19+ B cells (SO = 20.1 ± 6.7 vs. CO = 17.1 ± 6.1%, p = 0.03), despite lower absolute numbers (SO = 0.57 ± 0.20 vs. CO = 0.63 ± 1.9 g/L, p < 0.01). However, no difference in the lymphocyte profile was found between children with SO and those with the most severe CO (BMI Z score ≥ 4.7 SD). CONCLUSION: Children with SO have altered blood lymphocyte profiles with increased prevalence of CD19+ B cells, which is closely linked to the degree of obesity severity and inflammatory markers.


Asunto(s)
Obesidad Infantil , Niño , Humanos , Obesidad Infantil/epidemiología , Estudios Retrospectivos , Índice de Masa Corporal , Linfocitos
2.
Arch Pediatr ; 29(4): 312-325, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-35305879

RESUMEN

OBJECTIVES: Nutritional vitamin D supplements are often used in general pediatrics. Here, the aim is to address vitamin D supplementation and calcium nutritional intakes in newborns, infants, children, and adolescents to prevent vitamin D deficiency and rickets in general populations. STUDY DESIGN: We formulated clinical questions relating to the following categories: the Patient (or Population) to whom the recommendation will apply; the Intervention being considered; the Comparison (which may be "no action," placebo, or an alternative intervention); and the Outcomes affected by the intervention (PICO). These PICO elements were arranged into the questions to be addressed in the literature searches. Each PICO question then formed the basis for a statement. The population covered consisted of children aged between 0 and 18 years and premature babies hospitalized in neonatology. Two groups were assembled: a core working group and a voting panel from different scientific pediatric committees from the French Society of Pediatrics and national scientific societies. RESULTS: We present here 35 clinical practice points (CPPs) for the use of native vitamin D therapy (ergocalciferol, vitamin D2 and cholecalciferol, vitamin D3) and calcium nutritional intakes in general pediatric populations. CONCLUSION: This consensus document was developed to provide guidance to health care professionals on the use of nutritional vitamin D and dietary modalities to achieve the recommended calcium intakes in general pediatric populations. These CPPs will be revised periodically. Research recommendations to study key vitamin D outcome measures in children are also suggested.


Asunto(s)
Neonatología , Deficiencia de Vitamina D , Adolescente , Calcio , Calcio de la Dieta , Niño , Preescolar , Colecalciferol , Consenso , Suplementos Dietéticos , Humanos , Lactante , Recién Nacido , Vitamina D/uso terapéutico , Deficiencia de Vitamina D/epidemiología , Deficiencia de Vitamina D/prevención & control , Vitaminas/uso terapéutico
3.
Arch Pediatr ; 28(1): 69-74, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33268182

RESUMEN

Recently, the European Commission issued a Delegated Regulation updating the compositional and information requirements for infant and follow-on formulae that are to be applied at the latest in February 2021. This new regulation changes the status of docosahexaenoic acid (DHA) from an optional ingredient to a mandatory nutrient in these formulae at levels between 20 and 50mg/100kcal (0.5-1% of fatty acids). By contrast, arachidonic acid (ARA) becomes an optional nutrient. Following publication of the new regulation, global scientific experts have expressed concerns regarding the potential health risks of new infant formulae containing only DHA, especially at levels higher than those in breast milk and infant formulae marketed to date. Both DHA and ARA play a crucial role in infant development. First, breast milk, the gold standard for infant feeding, contains both DHA and ARA. Second, during development, the conversion of linoleic acid into ARA through desaturation steps is not sufficient to meet nutritional needs, especially in carriers of newly identified genetic variants in fatty acid desaturases, which weaken the biosynthetic production of ARA. Third, circulating levels of DHA and ARA in breastfed infants can only be matched with the addition of both fatty acids to formulae. And fourth, most studies performed to date have demonstrated that important physiological and developmental endpoints are sensitive to the ratio of dietary ARA:DHA. The precautionary principle applies when implementing the new EU regulation for infant and follow-on formulae. As a consequence, given the vulnerability of developing infants as well as the absence of conclusive evidence that formulae with at least 20mg DHA/100kcal, but no ARA, are safe and suitable to support the growth and development of infants similar to their breastfed peers, it remains necessary to still market formulas containing both ARA and DHA until proved otherwise.


Asunto(s)
Ácido Araquidónico , Grasas de la Dieta , Fórmulas Infantiles/química , Fórmulas Infantiles/normas , Fenómenos Fisiológicos Nutricionales del Lactante , Ingesta Diaria Recomendada , Desarrollo Infantil , Ácidos Docosahexaenoicos , Europa (Continente) , Humanos , Lactante , Recién Nacido , Leche Humana/química
4.
Arch Pediatr ; 27(8): 490-496, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-32950332

RESUMEN

Iron deficiency and iron deficiency anemia are public health issues recognised by the World Health Organisation, especially in sub-Saharan Africa. In some countries on this continent, the prevalence of anemia exceeds 60% in the pediatric population. Iron supplementation could prevent around one third of anemia cases in children in Africa and could decrease morbidity and mortality. A number of factors are behind this anemia, including iron deficiency caused by an inadequate diet, pica and geophagia, as well as chronic inflammation (malaria, digestive and urinary parasites, etc.). Ferritin is a good indicator of the body's iron stores, but it is not a specific reflection, with both inflammation and infection causing an increase. Ferritin could be interpreted according to C-reactive protein (CRP) or alpha-1 glycoprotein (AGP), or by adjusting the ferritin threshold to 30µg/L. The treatment of malaria and digestive or urinary worms, the correction of factors that stimulate inflammation, the fight against geophagia, as well as more hygienic living conditions, are all prerequisites for overcoming iron deficiency. A number of campaigns using iron supplements have proven to be effective, especially in schools, to fight against iron deficiency and malnutrition. Biofortification is an innovative and promising cultivation technique that increases the content of bioavailable iron in local produce such as beans. It will become a lever in the fight against iron deficiency.


Asunto(s)
Anemia Ferropénica , África del Sur del Sahara/epidemiología , Anemia Ferropénica/epidemiología , Anemia Ferropénica/etiología , Anemia Ferropénica/prevención & control , Niño , Preescolar , Humanos , Lactante , Prevalencia , Factores de Riesgo
5.
Arch Pediatr ; 27(7): 368-371, 2020 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-32807620

RESUMEN

BACKGROUND: Allergic reactions to food additives are often suspected by families. The aim of this study was to describe oral food challenge (OFC) outcomes in a pediatric cohort with a suspected diagnosis of allergy to food additives (food dyes or sodium benzoate). METHODS: All patients who underwent an open OFC to carmine red, cochineal red, erythrosine, patent blue V, tartrazine, yellow sunset S, and/or sodium benzoate were included. A survey was sent to families after testing to evaluate whether the OFC results had altered feeding behaviors with food additives. RESULTS: Twenty-three patients were included. The main suspected food was candy (n=11/23; 48%). Only one OFC out of 45 was formally positive for the carmine and cochineal red. Subsequently, most OFCs were negative (44/45; 97.8%). Despite the negativity of the challenge, four families out of 14 reported occurrences of supposed allergic reactions to food additives and six out of 15 continued to completely avoid the additive of concern in their children's diet. CONCLUSIONS: Allergies to food additives remain rare. Even if an IgE-mediated allergy was excluded with a negative OFC, families remained suspicious about ready-made products. Health professionals and parents should be reassured about the low risk of food dye intolerance or allergies.


Asunto(s)
Colorantes de Alimentos/efectos adversos , Hipersensibilidad a los Alimentos/diagnóstico , Hipersensibilidad a los Alimentos/etiología , Conservantes de Alimentos/efectos adversos , Benzoato de Sodio/efectos adversos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Encuestas Epidemiológicas , Humanos , Lactante , Masculino , Estudios Prospectivos , Estudios Retrospectivos
6.
Arch Pediatr ; 27(4): 219-222, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-32334899

RESUMEN

We report the case of a 13-month-old infant who was referred to the pediatric emergency department because of psychomotor regression with four bone fractures due to nutritional rickets. The reason was prolonged breastfeeding from a vegetarian mother followed by a vegan diet for the infant after weaning. Rickets is one of the many nutritional deficiencies that could affect infants fed vegan or vegetarian diets. These diets are a public health concern requiring adapted information that suggests alternative formulas made from rice or soy proteins and adapted supplementation after weaning.


Asunto(s)
Dieta Vegana/efectos adversos , Raquitismo/diagnóstico , Lactancia Materna , Dieta Vegetariana/efectos adversos , Humanos , Lactante , Masculino , Raquitismo/etiología , Destete
7.
Arch Pediatr ; 26(7): 442-450, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31615715

RESUMEN

The current craze for vegan diets has an effect on the pediatric population. This type of diet, which does not provide all the micronutrient requirements, exposes children to nutritional deficiencies. These can have serious consequences, especially when this diet is introduced at an early age, a period of significant growth and neurological development. Even if deficiencies have less impact on older children and adolescents, they are not uncommon and consequently should also be prevented. Regular dietary monitoring is essential, vitamin B12 and vitamin D supplementation is always necessary, while iron, calcium, docosahexaenoic acid, and zinc should be supplemented on a case-by-case basis.


Asunto(s)
Dieta Vegana/normas , Desnutrición/prevención & control , Adolescente , Niño , Dieta Vegana/efectos adversos , Dieta Vegana/métodos , Suplementos Dietéticos/normas , Francia , Humanos , Desnutrición/diagnóstico , Desnutrición/etiología
8.
Arch Pediatr ; 26(3): 179-181, 2019 Apr.
Artículo en Francés | MEDLINE | ID: mdl-30827774

RESUMEN

We report three pediatric cases of two, five, and 12 neodymium magnet spheres ingested simultaneously. In two cases, endoscopic extraction failed because of the strength of the magnetic attraction and a gastrotomy was performed to remove all magnets. In the third case, the magnets were not accessible endoscopically and were removed by laparotomy. We wish to warn consumer groups and pediatricians about the danger of neodymium magnets.


Asunto(s)
Ingestión de Alimentos , Cuerpos Extraños/diagnóstico por imagen , Imanes , Niño , Preescolar , Endoscopía del Sistema Digestivo , Cuerpos Extraños/cirugía , Humanos , Masculino , Neodimio
9.
Arch Pediatr ; 26(2): 86-94, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30638928

RESUMEN

INTRODUCTION: Psoriasis is associated with a higher risk of cardiovascular and/or metabolic comorbidity in adults, but discordant data have been reported in children. OBJECTIVE: To evaluate the frequency of metabolic and cardiovascular comorbidity in children with psoriasis and to establish whether age at onset of psoriasis correlates with metabolic and cardiovascular comorbidity in adulthood. MATERIAL AND METHODS: We conducted a systematic review on MEDLINE, using PubMed and Ovid. The search was limited to children (<18 years). The following key words were used: "psoriasis" with "children or childhood or adolescent" and "obesity" or "hypertension" or "diabetes" or "dyslipidemia" or "cardiovascular risk factor" or "myocardial infarction" or "stroke" or "coronaropathy" or "comorbidity". The reference lists of the articles retrieved were checked for additional relevant studies. RESULTS: A total of 377 potential citations were analyzed. After removing duplicate articles and reviewing eligibility in titles and abstracts, 16 articles remained. The studies analyzed revealed significantly higher risk of overweight and obesity in children with psoriasis, despite the numerous definitions used. Four studies reported higher risk of abdominal obesity in children with psoriasis. Data on hypertension, diabetes, dyslipidemia, metabolic syndrome, and major cardiovascular events suggested there was no higher risk of these comorbidities in children with psoriasis. Two studies suggested that age at onset of psoriasis did not increase the frequency of comorbidity in adulthood. CONCLUSION: This systematic review suggests that psoriasis in children is not associated with metabolic and cardiovascular comorbidities, except overweight and obesity, for which higher prevalence is clearly demonstrated in the literature.


Asunto(s)
Enfermedades Cardiovasculares/epidemiología , Enfermedades Metabólicas/epidemiología , Psoriasis/epidemiología , Adulto , Edad de Inicio , Niño , Comorbilidad , Salud Global , Humanos
10.
Arch Pediatr ; 24(10): 1000-1004, 2017 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-28927774

RESUMEN

BACKGROUND: Treacher Collins syndrome (TCS) mainly presents with severe craniofacial developmental abnormalities characterized by a combination of bilateral downward-slanting palpebral fissures, colobomas of the lower eyelids, hypoplasia of the facial bones, cleft palate, malformation of the external ears, atresia of the external auditory canals, and bilateral conductive hearing loss. It is due to mutations in Treacher Collins syndrome 1 (TCOF1) (5q32-q33.1) and Polymerase RNA 1 polypeptides D and C (POLR1D [13q12.2], and POLR1C [6p21.1]) genes, which are responsible for increased neuroepithelial apoptosis during embryogenesis resulting in the lack of neural crest cells involved in facial bone and cartilage formation. Altered function of the upper digestive tract has been reported, whereas severe dysmotility disorders have never been reported. We describe here the first case of TCS associated with histologically proven chronic intestinal pseudo-obstruction (CIPO) in humans. Case presentatios A 12-year-old boy with TCS due to TCOF1 gene deletion experienced nutritional difficulties and digestive intolerance from birth. CIPO was suspected during childhood because of severe intestinal dysmotility leading to enteral-jejunal nutrition intolerance and dependence on total parenteral nutrition. Diagnosis of CIPO with nervous abnormalities was histologically confirmed on a surgical rectal biopsy that showed enlarged ganglionic myenteric plexus. At the age of 9 years, an isolated colonic stenosis without dilatation responsible for severe abdominal pain and altered quality of life led to digestive derivation contributing to rapid disappearance of chronic abdominal pain. At the age of 12 years, the patient was still dependent on total home parenteral nutrition 7 days a week to maintain regular growth velocity. CONCLUSION: Recently, mice studies have pointed out the role played by TCOF1 in ganglionic cell migration in the foregut, suggesting that the synergistic haploinsufficiency of Tcof1 and Pax3, a transcription factor regulating the RET gene involved in disorders of neural crest cell development, probably results in colonic aganglionosis and may explain the association described here between TCS and CIPO. This case may correspond to this possible mechanism in humans. These findings and our clinical report suggest that CIPO may be assessed as unusual digestive manifestations in TCS with TCOF1 deletion.


Asunto(s)
Seudoobstrucción Intestinal/etiología , Disostosis Mandibulofacial/complicaciones , Niño , Enfermedad Crónica , Humanos , Masculino
11.
Arch Pediatr ; 24(5S): 5S23-5S31, 2017 May.
Artículo en Francés | MEDLINE | ID: mdl-28622779

RESUMEN

Iron deficiency is the main nutritional disease worldwide. The multidisciplinary working group of the French Pediatric Society on iron assessed the following recommendations. Since iron is variably absorbed depending on foods, the group defined absorbed iron requirements instead of dietary iron requirements. Iron-rich foods should be introduced at 4-months of age in some breast-fed infants. Between 7 and 11 month-old, formula-fed infants should drink at least 700ml per day of follow-on formula and partially or totally breast-fed infants should receive oral iron supplementation. Between 1 and 6 year-old, children should drink at least 300ml per day of growing-up milk until they become able to consume 100 to 150 grams per day of meat products. From 7 year-old, consumption of 2 portions per day of meat products is necessary in order to achieve daily iron requirements. Oral iron therapy from either increased iron-rich foods consumption or iron salts preparations is indicated when absorbed iron requirements are not achieved or when ferritin concentration is lower than age-specific limit values.


Asunto(s)
Fenómenos Fisiológicos Nutricionales Infantiles , Fenómenos Fisiológicos Nutricionales del Lactante , Hierro de la Dieta/administración & dosificación , Necesidades Nutricionales , Anemia Ferropénica/prevención & control , Niño , Suplementos Dietéticos , Humanos , Lactante , Absorción Intestinal , Deficiencias de Hierro
13.
Arch Pediatr ; 23(10): 1063-1066, 2016 Oct.
Artículo en Francés | MEDLINE | ID: mdl-27618291

RESUMEN

INTRODUCTION: Duodenal duplications are rare congenital malformations whose revealing signs are highly variable and nonspecific. OBSERVATION: We report the case of a female infant who presented with neonatal acute pancreatitis complicated by recurrent ascites, profound hypoalbuminemia responsible for pleural and pericardial effusions, revealing a duodenal duplication cyst. The unusual and original clinical presentation as well as the difficulty detecting the duplication radiologically delayed the diagnosis. A prolonged medical treatment with octreotide, albumin infusions, and exclusive parenteral nutrition led to an almost total disappearance of the ascites before surgery. The outcome was favorable after surgical removal of the duplication with 1 year of follow-up. CONCLUSION: The diagnosis of duodenal duplication can be difficult and it may be necessary to repeat the ultrasound examinations. Surgical resection is delicate, especially when there is an abundant pancreatic ascites. Therefore, an adequate prolonged medical treatment to reduce this ascites is recommended before the surgery.


Asunto(s)
Duodeno/anomalías , Pancreatitis/etiología , Ascitis/etiología , Duodeno/diagnóstico por imagen , Femenino , Humanos , Recién Nacido
14.
Arch Pediatr ; 23(6): 603-6, 2016 Jun.
Artículo en Francés | MEDLINE | ID: mdl-27133373

RESUMEN

Narcolepsy is a disabling disorder, characterized by excessive daytime sleepiness, irresistible sleep attacks, and partial or complete cataplexy. Many cases of obesity and precocious puberty have been reported in narcoleptic children, suggesting that the deficiency of hypocretin in narcolepsy could also be implicated in appetite stimulation. We report the observations of two young girls, who were referred for obesity and who developed narcolepsy accompanied by an abrupt weight gain. In both cases, specific drugs promoted wakefulness and overweight stabilization. Narcolepsy has to be suspected in sleepy obese children and not misdiagnosed as obstructive apnea. A nocturnal polysomnography with multiple sleep latency tests should be performed to confirm the diagnosis and begin specific treatment that is effective for sleep disorders and weight gain.


Asunto(s)
Narcolepsia/complicaciones , Narcolepsia/diagnóstico , Obesidad Infantil/complicaciones , Adolescente , Niño , Femenino , Humanos , Orexinas/análisis , Polisomnografía
15.
Arch Pediatr ; 22(12): 1233-9, 2015 Dec.
Artículo en Francés | MEDLINE | ID: mdl-26527542

RESUMEN

INTRODUCTION: In young obese patients, the transition from adolescence to adulthood, i.e., the transition from the pediatric to the adult medical team, is a new issue. In particular, it is important to define when and how this transition should be made in the setting of bariatric surgery. MATERIALS AND METHODS: Fourteen young obese patients (under the age of 20), who underwent bariatric surgery, were included in the study (nine cases of Roux-en-Y gastric by-pass, three sleeve gastrectomy, one gastric banding). After surgery, the patients were followed in both the pediatric and adult departments (protocol 1) or only in the pediatric department during the 1st year and then in the adult department afterwards (protocol 2). Anthropometric and metabolic data, before and after surgery, and compliance monitoring were analyzed using a retrospective design. Twelve patients completed a questionnaire assessing how they experienced the transition. RESULTS: Before surgery, mean age±SD was 16.3±1.8 years old and mean body mass index (BMI) 55.0±8.6kg/m(2). At 1 year after surgery, mean weight loss was -32.1±8.2% of initial body weight. Adherence to vitamin supplementation was judged to be adequate (vitamins were not taken less than once a week) for only 57.5% patients. Mean follow-up was 34.8±25.1 months [95% CI, 9.5-78.4]. None of the patients was lost to follow-up. Compliance was significantly better for patients following protocol 2. Adolescents reported being satisfied with meetings and newsletters about surgery, specific to this age group (91.7%). They also reported that information on the adult department was sufficient and 91.7% of them expressed satisfaction on the first outpatient visit in the adult department. However, all patients spontaneously reported having difficulties identifying members of the different teams: nutritionist pediatrician, nutritionist, and adult surgeon. DISCUSSION: These preliminary data suggest that, in obese adolescents, it is important to differentiate the transition period and the time and preparation for bariatric surgery. A prospective follow-up with a larger number of subjects and recommendations are needed to better define and improve the specific clinical management of obese adolescents transitioning to adulthood.


Asunto(s)
Cirugía Bariátrica , Obesidad Mórbida/cirugía , Obesidad Infantil/cirugía , Transición a la Atención de Adultos , Adolescente , Niño , Femenino , Humanos , Masculino , Estudios Retrospectivos , Encuestas y Cuestionarios , Adulto Joven
18.
Arch Pediatr ; 22(7): 769-77, 2015 Jul.
Artículo en Francés | MEDLINE | ID: mdl-26051270

RESUMEN

Eosinophilic gastrointestinal disorders are a heterogeneous group of disorders characterized by no specific digestive symptoms associated with dense eosinophilic inflammation of the gastrointestinal tract in the absence of known causes for such tissue eosinophilia. Among these diseases, eosinophilic gastroenteritis (EGE) and colitis (EC) are less common than esophagitis, but their incidence and prevalence have been increasing over the past decade due in part to increased disease recognition. The exact pathophysiology is not clear: EGE and EC are immune-mediated diseases implicating adaptive T-helper cell type 2 immunity. According to the site of eosinophilic infiltration, there is a wide spectrum of digestive symptoms ranging from food refusal, nausea, vomiting, abdominal pain, weight loss, gastrointestinal bleeding (anemia), protein loosing enteropathy, ascites, bowel obstruction or perforation for EGE and diarrhea ± bleeding for CE. Endoscopic lesions are not specific: friability, erythematous mucosa with superficial erosions, or ulceration is often observed. Histologically, markedly increased numbers of mucosal eosinophils are seen in biopsy specimens. However, no standards for the diagnosis of EGE or CE exist and few findings support the diagnosis: intraepithelial eosinophils, eosinophil crypt abscesses, and eosinophils in muscularis mucosa and/or submucosa. Other organs are not involved. The other causes of tissue eosinophilia (infections, inflammatory bowel diseases) should be excluded. Food allergy appears to play a central role in driving inflammation in EGE and CE, as evidenced by symptomatic improvement with initiation of food exclusion or elemental diets. Dietary treatment should be the first therapeutic option in children. If the elimination diet fails, corticosteroids are currently the best characterized treatment but appropriate duration is unknown and relapses are frequent. In severe forms, immunomodulators or biologic agents (anti-IL5, anti-IgE, or anti-TNFa) can potentially play a role in the treatment of EGE and CE.


Asunto(s)
Colitis , Enteritis , Eosinofilia , Gastritis , Colitis/diagnóstico , Colitis/fisiopatología , Colitis/terapia , Árboles de Decisión , Enteritis/diagnóstico , Enteritis/fisiopatología , Enteritis/terapia , Eosinofilia/diagnóstico , Eosinofilia/fisiopatología , Eosinofilia/terapia , Gastritis/diagnóstico , Gastritis/fisiopatología , Gastritis/terapia , Humanos
20.
Arch Pediatr ; 21(8): 879-81, 2014 Aug.
Artículo en Francés | MEDLINE | ID: mdl-24962783

RESUMEN

Infant small-bowel intussusceptions, most of the time idiopathic, may exceptionally reveal a severe digestive disease. We report the case of a 4-month-old infant who presented multiple and simultaneous ileal intussusceptions associated with severe acute gastroenteritis. Initially, the infant showed a protein-losing enteropathy with a clear alteration of the general state of health and undocumented fever, resistant to broad-spectrum antibiotic therapy. Skin and splenic nodules associated with hepatosplenomegaly and pancytopenia set in progressively. The etiologic evaluation led to the diagnosis of a Mycobacterium bovis BCG infection related to severe combined immune deficiency. The treatment consisted in anti-tuberculosis quadruple therapy in association with immunoglobin supplementation. Secondarily, the patient underwent gene therapy in a clinical trial. An early BCG vaccine in the first weeks of life, before the outbreak of infection revealing the immune deficiency, is a risk factor in triggering a disseminated BCG infection in immunodepressed infants. This clinical case is the first reported of severe combined immune deficiency revealed by small-bowel intussusceptions related to a disseminated BCG infection.


Asunto(s)
Enfermedades del Íleon/microbiología , Intususcepción/microbiología , Mycobacterium bovis , Inmunodeficiencia Combinada Grave/complicaciones , Tuberculosis/etiología , Humanos , Lactante , Masculino , Recurrencia
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