RESUMEN
AIM: To investigate the correlation between capsule endoscopy (CE) classification of primary intestinal lymphangiectasia (PIL) and computed tomography (CT) lymphangiography (CTL). MATERIALS AND METHODS: A total of 52 patients with diagnosed PIL were enrolled. All patients were examined using CTL and small intestinal CE before surgery. CE assessments included the morphology, scope, colour, and size of lesions. CTL assessments included intestinal wall, lymphatic vessel dilatation, lymph fluid reflux, and lymphatic fistula. Patients were divided into three groups according to type diagnosed by CE, and the CTL characteristics were analysed among the groups. RESULTS: CE showed 15 patients with type I, 27 with II, and 10 with type III. Intestinal wall thickening was observed in 15 type I, 21 type II, and seven type III. Pericardial effusion was observed in only three type I patients; the difference among types was statistically significant (p=0.02). Abnormal contrast agent distribution in the intestinal wall and mesentery was observed in 15 type II patients, and the difference was significantly greater than that of types I and III (p=0.02). Abnormal contrast agent distribution in the abdominal cavity was observed in 12 type II, and the difference was statistically significant (p=0.03). CONCLUSION: The CE PIL classification reflects the extent and scope of intestinal mucosa lesions; CTL more systematically demonstrates abnormal lymphatic vessels or reflux, and its manifestations of PIL are related to the CE classification. The combination of CTL with CE is useful for accurately evaluating PIL, and provides guidance for preoperative assessment and treatment management of PIL patients.
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Endoscopía Capsular , Linfangiectasia Intestinal , Humanos , Linfangiectasia Intestinal/diagnóstico por imagen , Linfangiectasia Intestinal/patología , Linfografía/métodos , Medios de Contraste , Tomografía Computarizada por Rayos X/métodosRESUMEN
BACKGROUND: The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia (PIL). AIM: To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine the patients' imaging and pathological characteristics of the patients, and explain their causes. METHODS: We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1, 2007 and December 31, 2018. We compared and analyzed all endoscopic images, classified them into four types according to the endoscopic features of the intestinal mucosa, and analyzed the post-lymphographic computed tomography (PLCT) and pathological characteristics of each type. RESULTS: According to the endoscopic features of PIL in 123 patients observed during endoscopy, they were classified into four types: nodular-type, granular-type, vesicular-type, and edematous-type. PLCT showed diffuse thickening of the small intestinal wall, and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular types. Contrast agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous types. Analysis of the small intestinal mucosal pathology revealed that nodular-type and granular-type lymphangiectasia involved the small intestine mucosa in four layers, whereas ectasia of the vesicular- and edematous-type lymphatic vessels largely involved the lamina propria mucosae, submucosae, and muscular layers. CONCLUSION: Endoscopic classification, combined with the patients' clinical manifestations and pathological examination results, is significant and very useful to clinicians when scoping patients with suspected PIL.
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Linfangiectasia Intestinal , Edema/etiología , Endoscopía Gastrointestinal/efectos adversos , Humanos , Intestino Delgado/patología , Linfangiectasia Intestinal/diagnóstico por imagen , Linfangiectasia Intestinal/patología , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodosRESUMEN
Defects in the PIEZO1 gene cause lymphatic dysplasia in an autosomal recessive manner, mostly by loss-of-function variants. Moreover, since 2019, the role of PIEZO1 in bone formation has been established, but there have been no PIEZO1-related cases presenting definite skeletal involvement to date. A 21-year-old male with primary lymphatic dysplasia had some other distinctive clinical features, including multiple fracture history during infancy, thoracolumbar scoliosis, short stature, and left-sided facial bone hypoplasia. We analyzed the whole exome of the patient and found two novel pathogenic variants of PIEZO1 in trans: a 93.7 kb heterozygous deletion (chr16:88,782,477-88,876,207; exon 1-50) and c.2858G>A (p.Arg953His). Sanger sequencing validated the deletion with breakpoints, and each variant was inherited from a different parent. This study presented an extremely rare case of a patient with lymphatic dysplasia caused by compound heterozygous variants of PIEZO1, along with additional clinical manifestations including several skeletal phenotypes.
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Anomalías Craneofaciales/genética , Fracturas Óseas/genética , Canales Iónicos/genética , Linfangiectasia Intestinal/genética , Linfedema/genética , Mutación , Fenotipo , Escoliosis/genética , Anomalías Craneofaciales/patología , Fracturas Óseas/patología , Heterocigoto , Humanos , Linfangiectasia Intestinal/patología , Linfedema/patología , Masculino , Escoliosis/patología , Adulto JovenRESUMEN
Primary intestinal lymphangiectasia is an unusual cause of protein losing enteropathy due to either congenital malformation or obstruction of lymphatics of intestine. The disease can affect all or only a small part of the small intestine. Peripheral lymphedema may be associated. The diagnosis is based on endoscopic and histopathological findings. A 30-year-old woman presents lower extremity edema with hypoproteinemia, hypoalbuminemia, and hypogammaglobulinemia. Tc-labeled human serum albumin nanocolloid lymphoscintigraphy of the lower extremity demonstrated a dermal backflow in the right extremity consistent with lymphedema and an unusual ileal uptake on SPECT/CT. Diagnosis is confirmed on histopathological evaluation of biopsy of ileum.
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Linfangiectasia Intestinal/diagnóstico por imagen , Linfangiectasia Intestinal/patología , Compuestos de Organotecnecio , Albúmina Sérica , Tomografía Computarizada por Tomografía Computarizada de Emisión de Fotón Único , Adulto , Biopsia , Femenino , Humanos , Linfangiectasia Intestinal/complicaciones , Sensibilidad y EspecificidadRESUMEN
Generalized lymphatic dysplasia (GLD), characterized by lymphedema, lymphangiectasias, chylothorax, effusions, represents a recognized cause of fetal hydrops. We describe for the first time recurrent pregnancies showing different ultrasound presentations of lymphatic dysplasia. The first fetus displayed diffuse subcutaneous cysts and septations while the second one presented fetal hydrops. Exome sequencing results at 18 gestational weeks in the second pregnancy showed compound heterozygosity for two novel PIEZO1 variants, afterwards detected also in the first fetus and in the heterozygous parents. Both ultrasound and genetic findings expand the current knowledge of PIEZO1-related GLD. We suggest exome sequencing in hydropic fetuses with normal cytogenetics and in pregnancies with recurrent hydrops/lymphatic dysplasia.
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Anomalías Craneofaciales/genética , Pruebas Genéticas , Hidropesía Fetal/genética , Canales Iónicos/genética , Linfangiectasia Intestinal/genética , Linfedema/genética , Ultrasonografía Prenatal , Adulto , Anomalías Craneofaciales/diagnóstico por imagen , Anomalías Craneofaciales/patología , Femenino , Humanos , Hidropesía Fetal/diagnóstico por imagen , Hidropesía Fetal/patología , Linfangiectasia Intestinal/diagnóstico por imagen , Linfangiectasia Intestinal/patología , Linfedema/diagnóstico por imagen , Linfedema/patología , Embarazo , Secuenciación del ExomaRESUMEN
The etiology of nonimmune hydrops fetalis is extensive and includes genetic disorders. We describe a term-born female neonate with late onset extensive nonimmune hydrops, that is, polyhydramnios, edema, and congenital bilateral chylothorax. This newborn was successfully treated with repetitive thoracocentesis, total parenteral feeding, octreotide intravenously and finally surgical pleurodesis and corticosteroids. A genetic cause seemed plausible as the maternal history revealed a fatal nonimmune hydrops fetalis. A homozygous truncating variant in GDF2 (c.451C>T, p.(Arg151*)) was detected with exome sequencing. Genetic analysis of tissue obtained from the deceased fetal sibling revealed the same homozygous variant. The parents and two healthy siblings were heterozygous for the GDF2 variant. Skin and lung biopsies in the index patient, as well as the revised lung biopsy of the deceased fetal sibling, showed lymphatic dysplasia and lymphangiectasia. To the best of our knowledge, this is the first report of an association between a homozygous variant in GDF2 with lymphatic dysplasia, hydrothorax and nonimmune hydrops fetalis.
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Anomalías Craneofaciales/genética , Factor 2 de Diferenciación de Crecimiento/genética , Hidropesía Fetal/genética , Linfangiectasia Intestinal/genética , Linfedema/genética , Polihidramnios/genética , Anomalías Craneofaciales/diagnóstico , Anomalías Craneofaciales/patología , Femenino , Homocigoto , Humanos , Hidropesía Fetal/diagnóstico , Hidropesía Fetal/patología , Recién Nacido , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/patología , Linfedema/diagnóstico , Linfedema/patología , Polihidramnios/diagnóstico , Polihidramnios/patología , Embarazo , Toracocentesis , Ultrasonografía Prenatal , Secuenciación del ExomaRESUMEN
Hennekam lymphangiectasia-lymphedema syndrome is an autosomal recessive disorder characterized by congenital lymphedema, intestinal lymphangiectasia, facial dysmorphism, and variable intellectual disability. Known disease genes include CCBE1, FAT4, and ADAMTS3. In a patient with clinically diagnosed Hennekam syndrome but without mutations or copy-number changes in the three known disease genes, we identified a homozygous single-exon deletion affecting FBXL7. Specifically, exon 3, which encodes the F-box domain and several leucine-rich repeats of FBXL7, is eliminated. Our analyses of databases representing >100,000 control individuals failed to identify biallelic loss-of-function variants in FBXL7. Published studies in Drosophila indicate Fbxl7 interacts with Fat, of which human FAT4 is an ortholog, and mutation of either gene yields similar morphological consequences. These data suggest that FBXL7 may be the fourth gene for Hennekam syndrome, acting via a shared pathway with FAT4.
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Anomalías Craneofaciales/genética , Proteínas F-Box/genética , Predisposición Genética a la Enfermedad , Linfangiectasia Intestinal/genética , Linfedema/genética , Proteínas ADAMTS/genética , Alelos , Animales , Preescolar , Anomalías Craneofaciales/complicaciones , Anomalías Craneofaciales/patología , Drosophila melanogaster/genética , Genotipo , Homocigoto , Humanos , Linfangiectasia Intestinal/complicaciones , Linfangiectasia Intestinal/patología , Linfedema/complicaciones , Linfedema/patología , Masculino , Técnicas de Diagnóstico Molecular/métodos , Mutación/genética , Linaje , Fenotipo , Procolágeno N-Endopeptidasa/genéticaAsunto(s)
Enfermedades Duodenales/diagnóstico , Neoplasias Duodenales/diagnóstico , Linfangiectasia Intestinal/diagnóstico , Linfoma Folicular/diagnóstico , Anciano , Diagnóstico Diferencial , Enfermedades Duodenales/patología , Endoscopía del Sistema Digestivo , Femenino , Humanos , Linfangiectasia Intestinal/patología , Imagen de Banda EstrechaRESUMEN
OBJECTIVE: Intestinal lymphangiectasia (IL; primary or secondary) is an important cause of protein-losing enteropathy. We evaluated the clinicolaboratory profile, response to therapy, complications, and outcome of children with primary IL (PIL). METHODS: Consecutive children (≤18 years) diagnosed with PIL (clinical setting, typical small bowel histopathology, and exclusion of secondary causes) from 2007 to 2017 were evaluated. RESULTS: Twenty-eight children with PIL (16 boys, age at symptom onset-12 [1-192] months and at diagnosis 8 [1-18] years) were studied. Pedal edema (93%), chronic diarrhea (78.6%), and recurrent anasarca (64%) were the common presentations. Ascites, pleural, and pericardial effusion were seen in 64 (n-18; chylous-5, non-chylous-13), 18, and 18% cases, respectively. Hypoproteinemia, hypoalbuminemia, hypocalcemia, and lymphopenia were present in 82, 82, 75 and 39% cases, respectively. Duodenal biopsy established the diagnosis in 86% cases, while 14% required distal small bowel biopsies. Dietary therapy was given in all and 6 cases required additional therapy (octreotide-6, tranexamic acid-3, and total parenteral nutrition-1). Lymphedema (3/5 vs. 1/23), pleural effusion (4/5 vs. 1/23), and the need for additional therapy (4/5 vs. 2/23) were significantly more in patients with chylous ascites (n = 5) than those without chylous ascites (n = 23). Twenty-four cases in follow-up (39 [6-120] months) showed improvement; however, 8 required readmission (symptom recurrence-6 [25%], complication-2 [8.3%], Budd Chiari Syndrome-1, and abdominal B cell lymphoma-1). CONCLUSION: Presence of chylous ascites suggests severe disease in children with PIL. Majority of PIL children respond to dietary therapy; only 20% need additional therapy. Long-term follow-up is essential to monitor for symptoms relapse and complications.
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Linfangiectasia Intestinal/patología , Linfangiectasia Intestinal/terapia , Adolescente , Niño , Preescolar , Dieta , Endoscopía , Femenino , Humanos , Lactante , Recién Nacido , Intestino Delgado/patología , Linfangiectasia Intestinal/complicaciones , Linfangiectasia Intestinal/diagnóstico por imagen , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVES: Loss of the complement inhibitor CD55 leads to a syndrome of early-onset protein-losing enteropathy (PLE), associated with intestinal lymphangiectasia and susceptibility to large-vein thrombosis. The in vitro and short-term treatment benefits of eculizumab (C5-inhibitor) therapy for CD55-deficiency have been previously demonstrated. Here we present the 18-months treatment outcomes for 3 CD55-deficiency patients with sustained therapeutic response. METHODS: Three CD55-deficiency patients received off-label eculizumab treatment. Clinical and laboratory treatment outcomes included frequency and consistency of bowl movements, weight, patient/parent reports of overall well-being, and serum albumin and total protein levels. Membrane attack complex deposition on leukocytes was tested by flow cytometry, before and during eculizumab treatment. RESULTS: Marked clinical improvement was noted in all 3 patients with resolution of PLE manifestations, that is, diarrhea, edema, malabsorption, overall well-being, growth, and quality of life. In correlation with the clinical observations, we observed progress in all laboratory outcome parameters, including increase in albumin and total protein levels, and up to 80% reduction in membrane attack complex deposition on leukocytes (P < 0.001). The progress persisted over 18 months of treatment without any severe adverse events. CONCLUSIONS: CD55-deficiency patients present with early-onset diarrhea, edema, severe hypoalbuminemia, abdominal pain, and malnutrition. Targeted therapy with the terminal complement inhibitor eculizumab has positive clinical and laboratory outcomes in PLE related to CD55 loss-of-function mutations, previously a life-threatening condition. Our results demonstrate the potential of genetic diagnosis to guide tailored treatment, and underscore the significant role of the complement system in the intestine.
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Anticuerpos Monoclonales Humanizados/administración & dosificación , Antígenos CD55/deficiencia , Inactivadores del Complemento/administración & dosificación , Enteropatías Perdedoras de Proteínas/tratamiento farmacológico , Adulto , Niño , Preescolar , Ensayos de Uso Compasivo , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Estudios de Seguimiento , Humanos , Infusiones Intravenosas , Linfangiectasia Intestinal/complicaciones , Linfangiectasia Intestinal/patología , Uso Fuera de lo Indicado , Estudios Prospectivos , Enteropatías Perdedoras de Proteínas/etiología , Calidad de Vida , Inducción de RemisiónAsunto(s)
Inmunodeficiencia Variable Común/diagnóstico , Linfangiectasia Intestinal/diagnóstico , Linfedema/diagnóstico , Infecciones Bacterianas/etiología , Niño , Errores Diagnósticos , Edema/etiología , Femenino , Humanos , Linfangiectasia Intestinal/complicaciones , Linfangiectasia Intestinal/dietoterapia , Linfangiectasia Intestinal/patología , Linfedema/complicaciones , Linfedema/dietoterapia , Linfedema/patología , Enteropatías Perdedoras de Proteínas/etiología , Tomografía Computarizada por Tomografía Computarizada de Emisión de Fotón ÚnicoRESUMEN
We report the first case of nonimmune hydrops fetalis (NIHF) associated with a recessive, in-frame deletion of V205 in the G protein-coupled receptor, Calcitonin Receptor-Like Receptor (hCALCRL). Homozygosity results in fetal demise from hydrops fetalis, while heterozygosity in females is associated with spontaneous miscarriage and subfertility. Using molecular dynamic modeling and in vitro biochemical assays, we show that the hCLR(V205del) mutant results in misfolding of the first extracellular loop, reducing association with its requisite receptor chaperone, receptor activity modifying protein (RAMP), translocation to the plasma membrane and signaling. Using three independent genetic mouse models we establish that the adrenomedullin-CLR-RAMP2 axis is both necessary and sufficient for driving lymphatic vascular proliferation. Genetic ablation of either lymphatic endothelial Calcrl or nonendothelial Ramp2 leads to severe NIHF with embryonic demise and placental pathologies, similar to that observed in humans. Our results highlight a novel candidate gene for human congenital NIHF and provide structure-function insights of this signaling axis for human physiology.
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Secuencia de Aminoácidos , Proteína Similar al Receptor de Calcitonina , Anomalías Craneofaciales , Hidropesía Fetal , Linfangiectasia Intestinal , Linfedema , Ratones Transgénicos , Eliminación de Secuencia , Animales , Proteína Similar al Receptor de Calcitonina/genética , Proteína Similar al Receptor de Calcitonina/metabolismo , Anomalías Craneofaciales/genética , Anomalías Craneofaciales/metabolismo , Anomalías Craneofaciales/patología , Modelos Animales de Enfermedad , Femenino , Células HEK293 , Heterocigoto , Homocigoto , Humanos , Hidropesía Fetal/genética , Hidropesía Fetal/metabolismo , Hidropesía Fetal/patología , Linfangiectasia Intestinal/genética , Linfangiectasia Intestinal/metabolismo , Linfangiectasia Intestinal/patología , Linfedema/genética , Linfedema/metabolismo , Linfedema/patología , Masculino , Ratones , Placenta , EmbarazoRESUMEN
RATIONALE: Primary intestinal lymphangiectasia (PIL) is a rare, presumably congenital lesion that is usually diagnosed in patients <â3 years old, is rarely first diagnosed in adulthood, and when first diagnosed in adulthood typically presents with symptoms for many years. Although PIL is often identified by endoscopic abnormalities, it must be emphasized that the jejunoileum/distal duodenum must be intubated for diagnosis because the lesions are present in these regions. This work demonstrates that 1)-PIL can occur in an octogenarian; 2)-shows that the characteristic endoscopic findings are not found at colonoscopy without terminal ileal intubation; and 3)-may be missed at standard EGD without distal duodenal intubation. DIAGNOSES: A patient initially presented at age 83 with symptoms of watery diarrhea, abdominal distention, 5-Kg-weight-gain, and weakness for one month, and had typical clinical findings of PIL including chylous ascites, pleural effusions, bilateral pitting leg edema, hypoalbuminemia, borderline lymphopenia, hypovitaminosis-D, and hypocalcemia. Protein-losing-enteropathy was demonstrated by positive stool tests for alpha-1-antitrypsin. Standard colonoscopy revealed no significant lesions, but terminal ileal intubation during colonoscopy demonstrated creamy-white, punctate, mucosal lesions in terminal ileum, characteristic of lymphangiectasia. EGD with intubation to mid-descending duodenum revealed no significant lesions, but subsequent enteroscopy demonstrated lesions in distal duodenum/proximal jejunum similar to those in terminal ileum characteristic of lymphangiectasia. Histopathologic analysis of lesions of terminal ileum/distal duodenum demonstrated dilated mucosal vessels, confirmed as lymphatic vessels by immunohistochemistry. PIL was diagnosed after excluding secondary causes of intestinal lymphangiectasia. INTERVENTIONS/OUTCOMES: Patient placed on standard PIL diet: oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins, and avoiding long-chain fatty acids, with marked clinical improvement. LESSONS: This work shows that: 1)-standard EGD and colonoscopy may miss characteristic lesions of PIL, 2)-enteroscopy or terminal ileal intubation at colonoscopy may be required for the diagnosis because lesions are typically located in distal duodenum/jejunoileum; and 3)-PIL can first present in the very elderly even with symptoms of short duration.
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Enteroscopía de Doble Balón , Endoscopía del Sistema Digestivo , Linfangiectasia Intestinal/patología , Anciano de 80 o más Años , Colonoscopía , Femenino , Humanos , Íleon , Intubación GastrointestinalRESUMEN
Inflammatory bowel disease (IBD) has a complex pathophysiology with limited treatments. Structural and functional changes in the intestinal lymphatic system have been associated with the disease, with increased risk of IBD occurrence linked to a history of acute intestinal injury. To examine the potential role of the lymphatic system in inflammation recurrence, we evaluated morphological and functional changes in mouse mucosal and mesenteric lymphatic vessels, and within the mesenteric lymph nodes during acute ileitis caused by a 7-day treatment with dextran sodium sulfate (DSS). We monitored whether the changes persisted during a 14-day recovery period and determined their potential consequences on dendritic cell (DC) trafficking between the mucosa and lymphoid tissues. DSS administration was associated with marked lymphatic abnormalities and dysfunctions exemplified by lymphangiectasia and lymphangiogenesis in the ileal mucosa and mesentery, increased mesenteric lymphatic vessel leakage, and lymphadenopathy. Lymphangiogenesis and lymphadenopathy were still evident after recovery from intestinal inflammation and correlated with higher numbers of DCs in mucosal and lymphatic tissues. Specifically, a deficit in CD103+ DCs observed during acute DSS in the lamina propria was reversed and further enhanced during recovery. We concluded that an acute intestinal insult caused alterations of the mesenteric lymphatic system, including lymphangiogenesis, which persisted after resolution of inflammation. These morphological and functional changes could compromise DC function and movement, increasing susceptibility to further gastrointestinal disease. Elucidation of the changes in mesenteric and intestinal lymphatic function should offer key insights for new therapeutic strategies in gastrointestinal disorders such as IBD. NEW & NOTEWORTHY Lymphatic integrity plays a critical role in small intestinal homeostasis. Acute intestinal insult in a mouse model of acute ileitis causes morphological and functional changes in mesenteric and intestinal lymphatic vessels. While some of the changes significantly regressed during inflammation resolution, others persisted, including lymphangiogenesis and altered dendritic cell function and movement, potentially increasing susceptibility to the recurrence of gastrointestinal inflammation.
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Ileítis/patología , Íleon/patología , Mucosa Intestinal/patología , Ganglios Linfáticos/patología , Linfangiectasia Intestinal/patología , Linfangiogénesis , Vasos Linfáticos/patología , Animales , Antígenos CD/metabolismo , Movimiento Celular , Células Dendríticas/metabolismo , Células Dendríticas/patología , Sulfato de Dextran , Modelos Animales de Enfermedad , Ileítis/inducido químicamente , Ileítis/metabolismo , Íleon/metabolismo , Cadenas alfa de Integrinas/metabolismo , Mucosa Intestinal/metabolismo , Ganglios Linfáticos/metabolismo , Linfangiectasia Intestinal/inducido químicamente , Linfangiectasia Intestinal/metabolismo , Vasos Linfáticos/metabolismo , Masculino , Ratones Endogámicos C57BL , Factores de TiempoAsunto(s)
Yeyuno/patología , Extremidad Inferior/fisiopatología , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/patología , Diagnóstico Diferencial , Humanos , Hipoalbuminemia/diagnóstico , Linfedema/diagnóstico , Masculino , Persona de Mediana Edad , Papiloma/diagnóstico , Enteroscopia de Balón IndividualRESUMEN
Primary intestinal lymphangiectasia (PIL), Waldmann's disease, is a rare disorder of unknown etiology characterized by dilated intestinal lacteals leading to lymph leakage into the small-bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. The main symptom is bilateral lower limb edema. Edema may be moderate to severe including pleural effusion, pericarditis or ascites. Protein-losing enteropathy is confirmed by the elevated 24-h stool α1-antitrypsin clearance and diagnosis by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of biopsies. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Several B-cell lymphomas of the gastrointestinal tract or with extra-intestinal localizations were reported in PIL patients. A long-term strictly low-fat diet associated with medium-chain triglyceride and liposoluble vitamin supplementation is the cornerstone of PIL medical management. Octreotide, a somatostatin analog, have been proposed with an inconsistent efficacy in association with diet. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. A prolonged clinical and biological follow-up is recommended.