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Linfangiectasia intestinal primária
ORPHA:90362CID-10 · I89.0CID-11 · LB15.YOMIM 152800DOENÇA RARA

A linfangiectasia intestinal primária (PIL) é uma doença intestinal rara caracterizada por dilatação do lúmen intestinal que causa vazamento de linfa para o lúmen do intestino delgado. As manifestações clínicas incluem edema relacionado à hipoalbuminemia (enteropatia perdedora de proteínas), astenia, diarréia, linfedema e retardo de crescimento em crianças.

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Introdução

O que você precisa saber de cara

📋

A linfangiectasia intestinal primária (PIL) é uma doença intestinal rara caracterizada por dilatação do lúmen intestinal que causa vazamento de linfa para o lúmen do intestino delgado. As manifestações clínicas incluem edema relacionado à hipoalbuminemia (enteropatia perdedora de proteínas), astenia, diarréia, linfedema e retardo de crescimento em crianças.

Publicações científicas
205 artigos
Último publicado: 2026 Jan-Dec

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I89.0
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
5 sintomas
🩸
Sangue
3 sintomas
📏
Crescimento
2 sintomas
🛡️
Imunológico
2 sintomas
🧬
Pele e cabelo
2 sintomas
❤️
Coração
1 sintomas

+ 25 sintomas em outras categorias

Características mais comuns

90%prev.
Linfangiectasia intestinal
Muito frequente (99-80%)
90%prev.
Anemia
Muito frequente (99-80%)
90%prev.
Imunodeficiência combinada
Muito frequente (99-80%)
90%prev.
Anormalidade funcional do trato gastrointestinal
Muito frequente (99-80%)
90%prev.
Edema
Muito frequente (99-80%)
90%prev.
Morfologia anormal do vaso linfático
Muito frequente (99-80%)
42sintomas
Muito frequente (6)
Frequente (9)
Ocasional (18)
Muito raro (2)
Sem dados (7)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 42 características clínicas mais associadas, ordenadas por frequência.

Linfangiectasia intestinalIntestinal lymphangiectasia
Muito frequente (99-80%)90%
Anemia
Muito frequente (99-80%)90%
Imunodeficiência combinadaCombined immunodeficiency
Muito frequente (99-80%)90%
Anormalidade funcional do trato gastrointestinalFunctional abnormality of the gastrointestinal tract
Muito frequente (99-80%)90%
Edema
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico205PubMed
Últimos 10 anos105publicações
Pico201716 papers
Linha do tempo
2026Hoje · 2026📈 2017Ano de pico🧪 2018Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
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·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥈Melhor nível de evidência: Observacional
Timeline de publicações
106 papers (10 anos)
#1

Primary intestinal lymphangiectasia presenting with intussusception in a child.

La Pediatria medica e chirurgica : Medical and surgical pediatrics2026 Feb 02

Intestinal lymphangiectasia is a protein losing intestinal disease caused by congenital malformation or obstruction of intestinal lymphatics. Based on etiology, it is classified as primary or secondary. Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of intestinal lymphatic dysfunction and may present with a wide spectrum of clinical manifestations but it very infrequently presents as intussusception in children. To the best of our knowledge this is the second only reported case in children, both cases being described from the Indian subcontinent. A 12-year-old male presented complaining of abdominal pain for one week. Examination revealed a palpable mass in the right upper quadrant and abdominal tenderness. A provisional diagnosis of acute intestinal obstruction was made and the child underwent an emergency laparotomy which revealed a segment of distal ileum displaying intussusception. On cut opening, no growth was seen. Histopathology of the telescoped segment showed many ectatic and dilated lymphatic channels filled with lymph. PIL should be considered in the differential diagnosis in children presenting with intestinal obstruction.

#2

Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.

Journal of pediatric gastroenterology and nutrition2026 Feb

Primary intestinal lymphangiectasia (PIL) is a very rare disease responsible for protein-losing enteropathy. There is little published data about treatments efficacy and outcomes. Our main objective was to describe the clinical profile, response to therapy, and outcomes of children with PIL. We conducted a national retrospective study including children with PIL followed in French university hospitals between 2010 and 2022. Response to treatment was defined as clinical remission and no need for albumin infusion. Response was considered complete if albuminemia was normal (>35 g/l) during follow-up and partial if less than 35 g/l. Thirty-four children (22 males) were included; median age at diagnosis was 7 (3-29,5) months. The median follow-up in our cohort was 4.5 years. Edema (79%), chronic diarrhea (50%), and ascites (35%) were the main symptoms. Thirty-one patients received a low long-chain triglycerides dietary therapy and 25 (81%) responded: 15 had a partial response and 10 a complete response. Four patients (12%) required a second-line drug treatment. The presence of lymphedema or an identified genetic variant were associated with a partial response to diet (p  < 0.05). A normal diet could be reintroduced in 14 patients (45%) without relapse during the follow-up. Among them, nine children (26%) were considered cured with a complete and prolonged remission under a normal diet. Most children with PIL responded to diet therapy and about a quarter of the cohort had a good prognosis with complete remission even after discontinuation of the diet. Presence of lymphedema or a genetic variant was associated with a chronic condition.

#3

An unusual etiology of diarrhea: primary intestinal lymphangiectasia.

Revista espanola de enfermedades digestivas2026 Feb

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by malformed and dilated intestinal lymphatic vessels, resulting in impaired drainage and leakage of lymph fluid into the gastrointestinal lumen. This leads to protein-losing enteropathy, causing chronic diarrhea, peripheral edema, weight loss, and hypoalbuminemia due to loss of proteins, lymphocytes, and lipids. Diagnosis relies on endoscopic visualization of pathognomonic white mucosal plaques (representing dilated lymphatics) and histopathological confirmation of lymphatic dilation, after excluding secondary causes like malignancy, surgery, or inflammatory conditions.

#4

Primary intestinal lymphangiectasia in a 5-month-old boy.

Clinical endoscopy2026 Jan
#5

Uncommon association of familial short stature with primary intestinal lymphangiectasia.

BMJ case reports2025 Dec 21

Primary intestinal lymphangiectasia (PIL) is a rare cause of lymphatic dysfunction leading to enteric protein loss in children. This case report describes a male child, with familial short stature who presented with chronic diarrhoea, anaemia, anasarca, lymphopaenia and hypoalbuminaemia due to PIL. Diagnosis was confirmed through a combination of endoscopic findings and histopathological analysis of small bowel biopsies showing dilated lymphatic vessels in the duodenum. The child responded to dietary intervention with medium chain triglycerides, fat soluble vitamin supplementations and subcutaneous octreotide therapy. The mainstay of management involves nutritional interventions with consideration of other supportive and therapeutic interventions.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC154 artigos no totalmostrando 105

2026

Primary intestinal lymphangiectasia presenting with intussusception in a child.

La Pediatria medica e chirurgica : Medical and surgical pediatrics
2025

Uncommon association of familial short stature with primary intestinal lymphangiectasia.

BMJ case reports
2026

Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.

Journal of pediatric gastroenterology and nutrition
2026

Primary intestinal lymphangiectasia in a 5-month-old boy.

Clinical endoscopy
2025

Clinical characterization of primary intestinal lymphangiectasia in infants and young children: a single-center retrospective analysis in China.

Translational pediatrics
2026

An unusual etiology of diarrhea: primary intestinal lymphangiectasia.

Revista espanola de enfermedades digestivas
2025

Primary intestinal lymphangiectasia: French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins).

Orphanet journal of rare diseases
2025

Elusive Edematous Primary Intestinal Lymphangiectasia in an Adult Patient Presenting With New Onset of Protein-Losing Enteropathy.

ACG case reports journal
2025

Secondary Hyperparathyroidism in Primary Intestinal Lymphangiectasia: A Report of Four Cases.

Gastroenterology research
2025

Intestinal lymphangiectasia in a dog: Clinical, ultrasonographic, endoscopic, and strain elastographic aspects.

Open veterinary journal
2025

Scintigraphic Improvement of Primary Intestinal Lymphangiectasia in a Patient Treated With Alpelisib for a PIK3CA-Related Overgrowth Spectrum.

Clinical nuclear medicine
2025

Elderly Onset Primary Intestinal Lymphangiectasia-A Rare Case.

JGH open : an open access journal of gastroenterology and hepatology
2025

Primary intestinal lymphangiectasia.

The lancet. Gastroenterology &amp; hepatology
2025

Lymphangiogram in primary intestinal lymphangiectasia.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2025

An evaluation of pozelimab for the treatment of CHAPLE disease.

Expert opinion on biological therapy
2024

[Clinical features of 50 patients with primary intestinal lymphangiectasia].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2024

Endoscopic Diagnosis of Primary Intestinal Lymphangiectasia in an Adult With Refractory Protein-Losing Enteropathy: A Case Report.

Cureus
2024

Primary intestinal lymphangiectasia - A rare cause of growth delay.

Pediatrics and neonatology
2024

Pharmacotherapy for CD55 deficiency with CHAPLE disease: how close are we to a cure?

Expert opinion on pharmacotherapy
2024

Primary intestinal lymphangiectasia: A case report.

Journal of pediatric gastroenterology and nutrition
2024

Waldmann's disease: Primary intestinal lymphangiectasia diagnosed by 99mTc-labeled albumin macroaggregate scintigraphy-A case report in an adult patient.

Clinical case reports
2024

Hypocalcaemic tetany linked to vitamin D deficiency and hypomagnesemia in primary intestinal lymphangiectasia: a literature review.

Annals of medicine and surgery (2012)
2024

Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital.

World journal of clinical cases
2023

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.

Frontiers in immunology
2024

Yellow Nail Syndrome: Analysis of 23 Consecutive Patients and Effect of Combined Fluconazole-Vitamin-E Treatment.

Dermatology (Basel, Switzerland)
2023

Newfound features associated with Hennekam Syndrome (Intestinal Lymphangiectasia-Lymphedema-Intellectual-Disability Syndrome) complicated with comorbid Waldmann's Disease resulting in Celiac Disease.

Clinical case reports
2024

Primary Intestinal Lymphangiectasia Successfully Controlled with a Denver Peritoneovenous Shunt for Refractory Ascites.

Internal medicine (Tokyo, Japan)
2023

A late diagnosis of primary intestinal lymphangiectasia in a Syrian girl.

Clinical case reports
2023

Misty mesentery, ascites, and bowel wall thickening in a child: Diagnostic clue for intestinal lymphangiectasia.

Radiology case reports
2023

Cryptococcal meningitis and cerebral vasculitis in a patient with primary intestinal lymphangiectasia: a case report.

European journal of clinical microbiology &amp; infectious diseases : official publication of the European Society of Clinical Microbiology
2023

Milky Way: Management of Primary Intestinal Lymphangiectasia.

Digestive diseases and sciences
2023

Colonic Lymphangiomatosis.

Cureus
2022

Adult-Onset Primary Intestinal Lymphangiectasia With Liver Enzymes Elevation.

ACG case reports journal
2023

Correlation between capsule endoscopy classification and CT lymphangiography of primary intestinal lymphangiectasia.

Clinical radiology
2022

Severe vaccine-acquired rotavirus infection in an infant with primary intestinal lymphangiectasia.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
2022

Endoscopic classification and pathological features of primary intestinal lymphangiectasia.

World journal of gastroenterology
2022

Primary intestinal lymphangiectasia presenting as limb convulsions: A case report.

World journal of clinical cases
2022

Diagnostic dilemma in diagnosing rare cause of protein losing enteropathy: Waldmann's disease.

Clinical case reports
2022

Protein-losing Enteropathy as a Complication and/or Differential Diagnosis of Common Variable Immunodeficiency.

Journal of clinical immunology
2022

Primary intestinal lymphangiectasia: a rare disease as a cause of protein-losing enteropathy.

Revista espanola de enfermedades digestivas
2021

A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma.

BMC gastroenterology
2021

Primary Intestinal Lymphangiectasia in a Middle-Aged Female.

Saudi journal of medicine &amp; medical sciences
2021

Primary intestinal lymphangiectasia in children: Twelve years of experience in the diagnosis and management.

Asia Pacific journal of clinical nutrition
2021

The Update of Treatment for Primary Intestinal Lymphangiectasia.

Pediatric gastroenterology, hepatology &amp; nutrition
2022

Dynamic Contrast Magnetic Resonance Lymphangiography Localizes Lymphatic Leak to the Duodenum in Protein-Losing Enteropathy.

Journal of pediatric gastroenterology and nutrition
2021

Complicated primary intestinal lymphangiectasia (Waldmann's disease) in a child successfully treated with octreotide: A case report from a low-resource setting.

Annals of medicine and surgery (2012)
2021

An unusual cause of heparin resistance - A case report.

Annals of cardiac anaesthesia
2021

Home parenteral nutrition a life-saving therapy in a primary intestinal lymphangiectasia patient affecting the entire GI tract - 3 year follow-up case report.

Annals of medicine and surgery (2012)
2021

Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review.

BMC gastroenterology
2021

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia.

Yonsei medical journal
2021

Refractory primary intestinal lymphangiectasia effectively managed with subcutaneous octreotide.

BMJ case reports
2021

Long-term endoscopic findings in pediatric primary intestinal lymphangiectasia.

Clinical case reports
2020

Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature.

World journal of gastroenterology
2021

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature.

BMC pediatrics
2021

Primary Intestinal Lymphangiectasia: Diagnostic Accuracy of 99mTc-Labeled Human Serum Albumin Nanocolloid SPECT/CT Before Biopsy.

Clinical nuclear medicine
2020

Primary intestinal lymphangiectasia diagnosed by video capsule endoscopy in a patient with immunodeficiency presenting with Morganella morganii bacteraemia.

BMJ case reports
2020

A case of Heck's disease with primary intestinal lymphangiectasia treated with imiquimod.

Indian journal of dermatology, venereology and leprology
2020

Tuberous sclerosis complex presenting as primary intestinal lymphangiectasia: A case report.

World journal of clinical cases
2020

Primary intestinal lymphangiectasia in children: A review.

Journal of paediatrics and child health
2020

Chronic diarrhea with white colon: primary intestinal lymphangiectasia.

QJM : monthly journal of the Association of Physicians
2020

First Report of Treatment with Pancreas-Sparing Duodenectomy in a Child with Primary Intestinal Lymphangiectasia.

Journal of Indian Association of Pediatric Surgeons
2019

Primary Intestinal Lymphangiectasia Causing Intussusception and Small Bowel Obstruction.

ACG case reports journal
2019

Primary intestinal lymphangiectasia in a 23-month- old girl.

Oxford medical case reports
2020

Protein-losing enteropathy and primary intestinal lymphangiectasia.

QJM : monthly journal of the Association of Physicians
2019

[Primary intestinal lymphangiectasia: first case report in Peru].

Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del Peru
2019

Clinical Profile, Response to Therapy, and Outcome of Children with Primary Intestinal Lymphangiectasia.

Digestive diseases (Basel, Switzerland)
2019

Pediatric localized intestinal lymphangiectasia treated with resection.

International medical case reports journal
2019

Comparative pathophysiology and management of protein-losing enteropathy.

Journal of veterinary internal medicine
2019

CHAPLE syndrome uncovers the primary role of complement in a familial form of Waldmann's disease.

Immunological reviews
2019

[Primary intestinal lymphangiectasia: Could propranolol be an effective alternative treatment?].

Anales de pediatria
2019

Novel Magnified Single-Balloon Enteroscopy Enables Observation of Jejunal White Spots Associated with Lymphangiectasia.

Digestive diseases (Basel, Switzerland)
2018

Milky Mesentery: Acute Abdomen with Chylous Ascites.

Indian pediatrics
2019

Primary Intestinal Lymphangiectasia (Waldmann's Disease).

The American journal of gastroenterology
2018

Primary intestinal lymphangiectasia and a review of the current literature.

The Turkish journal of gastroenterology : the official journal of Turkish Society of Gastroenterology
2019

CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE).

Academic radiology
2018

Case report of primary intestinal lymphangiectasia diagnosed in an octogenarian by ileal intubation and by push enteroscopy after missed diagnosis by standard colonoscopy and EGD.

Medicine
2017

Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports.

Medicine
2017

[Clinical analysis of intestinal lymphangiectasia in 47 children].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2017

[Primary intestinal lymphangiectasia associated with follicular lymphoma: one case report].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2017

Primary Intestinal Lymphangiectasia (Waldmann's Disease) Presenting with Chylous Effusions in a 15-Year-Old.

Journal of clinical and diagnostic research : JCDR
2017

[Diagnostic and therapeutic approach to chronic inflammatory enteropathies in dogs].

Tierarztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
2018

[Primary intestinal lymphangiectasia (Waldmann's disease)].

La Revue de medecine interne
2017

Exacerbation of primary intestinal lymphangiectasia during late pregnancy and recovery after delivery: A case report and literature review.

Medicine
2017

Primary intestinal lymphangiectasia in an elderly female patient: A case report on a rare cause of secondary immunodeficiency.

Medicine
2017

Cutaneous lymphangiectasia in a patient with primary intestinal lymphangiectasia.

International journal of dermatology
2017

Causes Of Chronic Non-Infectious Diarrhoea In Infants Less Than 6 Months Of Age: Rarely Recognized Entities.

Journal of Ayub Medical College, Abbottabad : JAMC
2017

CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis.

The New England journal of medicine
2017

Primary Intestinal Lymphangiectasia as a First Manifestation of Tuberous Sclerosis Complex.

Journal of pediatric gastroenterology and nutrition
2017

Waldmann's Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect.

Journal of clinical and diagnostic research : JCDR
2017

A primary intestinal lymphangiectasia hiding the diagnosis of pleural and pericardial tuberculosis: a clinical observation.

The Pan African medical journal
2017

Waldmann's disease: a rare cause of protein losing enteropathy in an adult patient.

Revista espanola de enfermedades digestivas
2017

Primary intestinal lymphangiectasia: Multiple detector computed tomography findings after direct lymphangiography.

Journal of medical imaging and radiation oncology
2017

Video Capsule Endoscopy to Diagnose Primary Intestinal Lymphangiectasia in a 14-Month-Old Child.

Journal of pediatric gastroenterology and nutrition
2016

Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report.

Medicine
2016

Secondary hypogammaglobulinemia in Waldmann's disease treated with subcutaneous immunoglobulins.

European annals of allergy and clinical immunology
2016

Everolimus for Primary Intestinal Lymphangiectasia With Protein-Losing Enteropathy.

Pediatrics
2015

Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report.

Journal of gastrointestinal and liver diseases : JGLD
2015

Primary intestinal lymphangiectasia with generalized warts.

World journal of gastroenterology
2015

Gastroenterology: Primary intestinal lymphangiectasia of the terminal ileum.

Journal of gastroenterology and hepatology
2015

Surgical therapy of primary intestinal lymphangiectasia in adults.

Journal of surgical case reports
2015

How to treat an extensive form of primary intestinal lymphangiectasia?

World journal of gastroenterology
2015

[Surgical treatment of rare combination of intestinal malrotation with secondary lymphangiectasia].

Vestnik Rossiiskoi akademii meditsinskikh nauk
2015

Primary intestinal lymphangiectasia treated with rapamycin in a child with tuberous sclerosis complex (TSC).

American journal of medical genetics. Part A
2015

A novel CCBE1 mutation leading to a mild form of hennekam syndrome: case report and review of the literature.

BMC medical genetics
2015

[Protein-losing enteropathy].

La Revue de medecine interne
Ver todos os 154 no EuropePMC

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Primary intestinal lymphangiectasia presenting with intussusception in a child.
    La Pediatria medica e chirurgica : Medical and surgical pediatrics· 2026· PMID 41685404mais citado
  2. Clinical presentation, treatment, and outcome of children with primary intestinal lymphangiectasia: A national retrospective study.
    Journal of pediatric gastroenterology and nutrition· 2026· PMID 41277165mais citado
  3. An unusual etiology of diarrhea: primary intestinal lymphangiectasia.
    Revista espanola de enfermedades digestivas· 2026· PMID 40737280mais citado
  4. Primary intestinal lymphangiectasia in a 5-month-old boy.
    Clinical endoscopy· 2026· PMID 41122770mais citado
  5. Uncommon association of familial short stature with primary intestinal lymphangiectasia.
    BMJ case reports· 2025· PMID 41423291mais citado
  6. Primary Intestinal Lymphangiectasia Presenting as Recurrent Chylous Ascites: A Rare Case.
    J Investig Med High Impact Case Rep· 2026· PMID 41943452recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:90362(Orphanet)
  2. OMIM OMIM:152800(OMIM)
  3. MONDO:0007916(MONDO)
  4. GARD:7873(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q7961531(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Linfangiectasia intestinal primária
Compêndio · Raras BR

Linfangiectasia intestinal primária

ORPHA:90362 · MONDO:0007916
Prevalência
Unknown
CID-10
I89.0 · Linfedema não classificado em outra parte
CID-11
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0024215
EuropePMC
Wikidata
Papers 10a
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