Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 33
Filtrar
1.
Eur J Clin Microbiol Infect Dis ; 42(10): 1263-1267, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37668805

RESUMO

Primary intestinal lymphangiectasia (Waldmann's disease) is a rare exudative enteropathy without precisely assessed infectious risk. We report the case of a 49-year-old male patient with meningitis and cerebral vasculitis due to Cryptococcus neoformans complicating Waldmann's disease diagnosed 12 years ago. The treatment combined liposomal amphotericin B, 3 mg/kg daily plus flucytosine 25 mg/kg/6 h, both intravenously during 15 days, then fluconazole 800 mg daily during 8 weeks, and finally 200 mg daily indefinitely. Dexamethasone 0.4 mg/kg daily during the first week was gradually decreased over 2 months. The outcome was good, and the patient is still followed 3 years later without any recurrence.


Assuntos
Criptococose , Cryptococcus neoformans , Meningite Criptocócica , Vasculite do Sistema Nervoso Central , Masculino , Humanos , Pessoa de Meia-Idade , Meningite Criptocócica/complicações , Meningite Criptocócica/diagnóstico , Meningite Criptocócica/tratamento farmacológico , Criptococose/complicações , Criptococose/diagnóstico , Criptococose/tratamento farmacológico , Vasculite do Sistema Nervoso Central/complicações , Vasculite do Sistema Nervoso Central/diagnóstico , Vasculite do Sistema Nervoso Central/tratamento farmacológico
2.
J Clin Immunol ; 42(7): 1461-1472, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35737255

RESUMO

As protein-losing enteropathy (PLE) can lead to hypogammaglobulinemia and lymphopenia, and since common variable immunodeficiency (CVID) is associated with digestive complications, we wondered if (1) PLE could occur during CVID and (2) specific features could help determine whether a patient with antibody deficiency has CVID, PLE, or both. Eligible patients were thus classified in 3 groups: CVID + PLE (n = 8), CVID-only (= 19), and PLE-only (n = 13). PLE was diagnosed using fecal clearance of α1-antitrypsin or 111In-labeled albumin. Immunoglobulin (Ig) A, G, and M, naive/memory B and T cell subsets were compared between each group. CVID + PLE patients had multiple causes of PLE: duodenal villous atrophy (5/8), nodular follicular hyperplasia (4/8), inflammatory bowel disease-like (4/8), portal hypertension (4/8), giardiasis (3/8), and pernicious anemia (1/8). Compared to the CVID-only group, CVID + PLE patients had similar serum Ig levels, B cell subset counts, but lower naive T cell proportion and IgG replacement efficiency index. Compared to the CVID-only group, PLE-only patients did not develop infections but had higher serum levels of IgG (p = 0.03), IgA (p < 0.0001), and switched memory B cells (p = 0.001); and decreased naive T cells (CD4+: p = 0.005; CD8+: p < 0.0001). Compared to the PLE-only group, CVID + PLE patients had higher infection rates (p = 0.0003), and lower serum Ig (especially IgA: p < 0.001) and switched memory B cells levels. In conclusion, PLE can occur during CVID and requires higher IgG replacement therapy dosage. PLE can also mimic CVID and is associated with milder immunological abnormalities, notably mildly decreased to normal serum IgA and switched memory B cell levels.


Assuntos
Imunodeficiência de Variável Comum , Enteropatias Perdedoras de Proteínas , Humanos , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Enteropatias Perdedoras de Proteínas/etiologia , Enteropatias Perdedoras de Proteínas/complicações , Diagnóstico Diferencial , Imunoglobulina A , Imunoglobulina G
3.
BMC Gastroenterol ; 21(1): 461, 2021 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-34895151

RESUMO

BACKGROUND: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma. CASE PRESENTATION: A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Laboratory investigations revealed lymphopenia, hypoalbuminemia, decreased triglyceride and cholesterol level. Colonoscopy showed multiple smooth pseudo polyps in the ileocecal valve and terminal ileum and histological examination showed conspicuous dilation of the lymphatic channels in the mucosa and submucosa. A diagnosis of PIL was made. Three years later colonoscopy of the patient showed an intraluminal proliferative mass in the ascending colon and biopsy examination confirmed a malignant non-Hodgkin lymphoma. Then the patient was been underwent chemotherapy, and his clinical condition is satisfactory. CONCLUSION: Our report supports the hypothesis that PIL is associated with lymphoma development.


Assuntos
Linfangiectasia Intestinal , Linfoma não Hodgkin , Enteropatias Perdedoras de Proteínas , Biópsia , Humanos , Linfangiectasia Intestinal/complicações , Linfócitos , Linfoma não Hodgkin/complicações , Masculino , Pessoa de Meia-Idade
4.
BMC Gastroenterol ; 21(1): 225, 2021 May 18.
Artigo em Inglês | MEDLINE | ID: mdl-34006223

RESUMO

BACKGROUND: Primary intestinal lymphangiectasia is an exceedingly rare disorder. Epidemiology is unknown. It usually presents with lower extremity swelling, diarrhea, ascites, and protein-losing enteropathy. Since the pathogenesis of edema is usually due to hypoalbuminemia; both extremities are typically involved. The edema can rarely be due to abnormal lymphatic circulation, causing lymphedema, which usually involves both extremities as well. Diagnosis is made by the constellation of clinical, biochemical, endoscopic, and histological findings. Treatment involves dietary modification, to reduce lymphatic dilation in response to dietary fat. Other pharmacologic (e.g., octreotide) and replacement measures may be indicated as well. The most serious long-term complication is intestinal lymphoma. Herein is a case of Primary intestinal lymphangiectasia presenting with unilateral lower limb swelling. CASE PRESENTATION: A 4-year-old boy presents with left foot swelling since the age of 4 months, in addition to intermittent diarrhea, and abdominal swelling. The foot swelling had been evaluated by different health care professionals in the past, and was mislabeled as either cellulitis, or congenital hemihyperplasia. Physical examination revealed mild ascites, and a non-pitting foot edema with a positive Stemmer's sign (lymphedema). Blood work revealed hypoalbuminemia (albumin 2 g/dl), and hypogammaglobulinemia. Endoscopy showed dilated lacteals throughout the duodenum. Histopathologic examination revealed massively dilated lamina propria lymphatics in the duodenal biopsies. The patient was diagnosed with primary intestinal lymphangiectasia. He was treated with high-protein and low-fat diet, and supplemental formula high in medium chain triglycerides. On follow-up, the patient's diarrhea completely resolved, and his ascites and edema improved significantly. CONCLUSIONS: The presence of unilateral lower limb edema should not preclude the diagnosis of systemic disorders, and a high index of suspicion is required in atypical presentations. A good knowledge about Primary intestinal lymphangiectasia manifestations, and physical examination skills to differentiate edema or lymphedema from tissue overgrowth can significantly aid in the diagnosis.


Assuntos
Linfangiectasia Intestinal , Linfedema , Enteropatias Perdedoras de Proteínas , Pré-Escolar , Extremidades , Humanos , Hiperplasia , Lactente , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/diagnóstico , Linfedema/diagnóstico , Linfedema/etiologia , Masculino , Enteropatias Perdedoras de Proteínas/diagnóstico , Enteropatias Perdedoras de Proteínas/etiologia
5.
BMC Pediatr ; 21(1): 21, 2021 01 07.
Artigo em Inglês | MEDLINE | ID: mdl-33407260

RESUMO

BACKGROUND: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. METHOD: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. RESULTS: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3-4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. CONCLUSION: Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.


Assuntos
Linfangiectasia Intestinal , Linfangiectasia , Adolescente , Criança , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/tratamento farmacológico , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/tratamento farmacológico , Imageamento por Ressonância Magnética , Estudos Prospectivos , Estudos Retrospectivos
6.
Dig Dis ; 37(2): 170-174, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30466073

RESUMO

A 59-year-old woman was diagnosed with primary intestinal lymphangiectasia (PIL), with characteristic findings on capsule enteroscopy and confirmation by histopathological examination of biopsy specimens. We viewed the abnormal jejunal mucosa using a newly developed magnifying single-balloon enteroscope (SIF-Y0007). Conventional observation showed leakage of chyle. However, using this new scope, we could see scattered white villi, representing dilated lymphatic vessels within the intestinal villi protruding from the dilated submucosal lymphoid vessels (D2-40 positive) within an edematous jejunal lesion. This report is the first to describe the white villi in a patient with PIL observed clearly using a newly developed magnifying enteroscope. Technological advancements and the accumulation of reported pathological data would further improve our understanding of the pathophysiological aspects of this disease entity, even in the jejunum.


Assuntos
Jejuno/patologia , Linfangiectasia Intestinal/diagnóstico , Enteroscopia de Balão Único , Duodeno/patologia , Feminino , Humanos , Jejuno/diagnóstico por imagem , Linfangiectasia Intestinal/diagnóstico por imagem , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
7.
J Ayub Med Coll Abbottabad ; 29(1): 78-82, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28712180

RESUMO

BACKGROUND: Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age. METHODS: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures were enrolled over a period of 8 months. Demographical profile and various factors under observation were recorded in this observational study. Collected data was analysed using SPSS version 20. Chi square test was applied as a test of significance for any qualitative variable, p value (p<0.05) was taken as significant. RESULTS: Among 72 enrolled patients, female to male ratio was1.05:1. Age at onset of symptoms was between 15 days to 6 months. Aetiology found was Cow's milk protein allergy (CMPA) in 58 (80.6%), Primary intestinal lymphangiectasia (PIL) 6 (8.3%), Cystic fibrosis (CF) 3 (4.2%), Immunodeficiency (SCID) 2 (2.8%), 1 (1.4%) for each Abetalipoproteinemia (ABL), Glucose galactose malabsorption (GGM) and Congenital chloride diarrhoea (CCD). CONCLUSIONS: Among noninfectious causes of chronic diarrhoea in early infancy, cow's milk protein allergy is most common followed by Primary intestinal lymphangiectasia and Cystic fibrosis.


Assuntos
Diarreia , Doença Crônica , Fibrose Cística , Diarreia/diagnóstico , Diarreia/etiologia , Feminino , Humanos , Lactente , Recém-Nascido , Linfangiectasia Intestinal , Linfedema , Masculino , Hipersensibilidade a Leite
8.
Am J Med Genet A ; 167A(9): 2209-12, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25943403

RESUMO

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by a congenital malformation of the lymphatic vessels of the small intestine causing insufficient drainage and leakage of lymph fluid. Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by benign hamartomas in multiple organ systems. While the lymphatic system has been implicated in TSC through lymphangioleiomyomatosis (LAM) and lymphedema, this paper reports the first case of PIL in TSC, a female patient with a TSC2 mutation. She developed persistent and significant abdominal distension with chronic diarrhea during her first year of life. Due to lack of treatment options and the involvement of the mTOR pathway in TSC, a trial of an mTOR inhibitor, rapamycin, was initiated. This treatment was highly effective, with improvement in clinical symptoms of PIL as well as abnormal laboratory values including VEGF-C, which was elevated to over seven times the normal upper limit before treatment. This case suggests that PIL is a rare manifestation of TSC, warranting the use of mTOR inhibitors in future studies.


Assuntos
Linfangiectasia Intestinal/tratamento farmacológico , Linfangiectasia Intestinal/genética , Linfedema/tratamento farmacológico , Linfedema/genética , Sirolimo/uso terapêutico , Esclerose Tuberosa/genética , Adulto , Feminino , Humanos , Mutação/genética , Proteína 2 do Complexo Esclerose Tuberosa , Proteínas Supressoras de Tumor/genética , Adulto Jovem
9.
Rev Gastroenterol Mex ; 79(1): 7-12, 2014.
Artigo em Espanhol | MEDLINE | ID: mdl-24655928

RESUMO

BACKGROUND: Primary intestinal lymphangiectasia is a rare congenital disease described by Waldmann in 1961 that is a consequence of obstruction of the lymphatic drainage of the small bowel with secondary lymph vessel dilation. This distorts the architecture of the villi and causes a leakage of lymph into the intestinal lumen, resulting in protein-losing enteropathy and malabsorption. AIM: To describe the clinical, biochemical, radiologic, endoscopic, and histologic characteristics in children with primary intestinal lymphangiectasia. METHOD: A retrospective observational, descriptive, cross-sectional study was conducted that reviewed the case records of children diagnosed with primary intestinal lymphangiectasia that were seen at the Department of Gastroenterology and Nutrition of the Instituto Nacional de Pediatría within the time frame of January 1, 1992 to September 30, 2012. RESULTS: Four patients were found that presented with primary intestinal lymphangiectasia. Three of them had been diagnosed before 3 years of age. All the patients presented with chronic diarrhea, edema, lymphopenia, hypocalcemia, and hypogammaglobulinemia, and 3 patients presented with hypocholesterolemia. Bowel transit time, endoscopy, and intestinal biopsies were characteristic of this pathology. CONCLUSIONS: Intestinal lymphangiectasia should be suspected when there is a clinical picture of chronic diarrhea and protein-losing enteropathy accompanied with edema at any level, as well as hypoalbuminemia, hypocalcemia, lymphopenia, hypogammaglobulinemia, and hypocholesterolemia, which are the main biochemical findings of this pathology. All children presenting with intestinal lymphangiectasia should undergo an upper gastrointestinal series with bowel transit time and endoscopy with biopsies taken at the level of the duodenum. Treatment includes diet and the periodic administration of albumin and gamma globulin.


Assuntos
Linfangiectasia Intestinal/terapia , Centros de Atenção Terciária , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Diarreia/diagnóstico , Diarreia/etiologia , Dieta , Endoscopia Gastrointestinal , Feminino , Humanos , Lactente , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/diagnóstico , Masculino , México , Estudos Retrospectivos
10.
Ann Med Surg (Lond) ; 86(4): 2049-2057, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38576918

RESUMO

Primary intestinal lymphangiectasia (PIL) is a rare disorder in children causing protein-losing enteropathy. Vitamin D deficiency and hypomagnesemia contributed to the tetany. The literature review reflects the importance of screening for these deficiencies and regular serum magnesium monitoring in PIL cases with neuromuscular or ionic abnormalities.

11.
World J Clin Cases ; 12(4): 746-757, 2024 Feb 06.
Artigo em Inglês | MEDLINE | ID: mdl-38322684

RESUMO

BACKGROUND: While primary intestinal lymphangiectasia (PIL) is considered a rare condition, there have been several reported cases in adults. Nevertheless, the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients. AIM: To enhance understanding by investigating clinical presentation, diagnosis, treatment, complications, and prognoses in adult PIL cases. METHODS: We enrolled adult patients diagnosed with PIL between March 2016 and September 2021. The primary outcome involved examining the diagnosis and treatment process of these patients. The secondary outcomes included identifying complications (infections, thromboembolism) and assessing prognoses (frequency of hospitalization and mortality) during the follow-up period. RESULTS: Among the 12 included patients, peripheral edema (100%) and diarrhea (75%) were the main presenting complaints. Laboratory tests showed that all the patients exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia. Radiologically, the predominant findings were edema of the small intestine (67%) and ascites (58%). The typical endoscopic finding with a snowflake appearance was observed in 75% of patients. Among the 12 patients, two responded positively to octreotide and sirolimus, and eight who could undergo maintenance therapy discontinued subsequently. Complications due to PIL led to infection in half of the patients, thromboembolism in three patients, and one death. CONCLUSION: PIL can be diagnosed in adults across various age groups, with different severity and treatment responses among patients, leading to diverse complications and prognoses. Consequently, tailored treatments will be necessary. We anticipate that our findings will contribute to the management of PIL, an etiology of protein-losing enteropathy.

12.
Clin Case Rep ; 12(4): e8772, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38634090

RESUMO

Key Clinical Message: Rare yet significant, this case sheds light on the uncommon presentation of Waldmann's disease in adults, showcasing the diagnostic challenges it poses. A multidisciplinary approach, integrating clinical, endoscopic, histological, and radiological evaluations, is crucial for accurate diagnosis and management. Further research is needed to deepen our understanding of this complex disorder. Abstract: Waldmann's disease, or primary intestinal lymphangiectasia, is a rare disorder characterized by protein-losing enteropathy due to dilation and leakage of intestinal lymphatic vessels. Although typically diagnosed in early childhood, we present a case of a 55-year-old male with a complex medical history. The patient's history included intestinal obstruction, multidrug-resistant pulmonary tuberculosis, and primary antiphospholipid syndrome. He presented with a 2-year history of chronic diarrhea, weight loss, and lower limb edema. Endoscopic and histological examination revealed scattered white spots in the duodenum and terminal ileum, indicative of intestinal lymphangiectasia. Nuclear medicine studies confirmed abnormal protein loss. The rarity of Waldmann's disease in adulthood and its association with other significant medical conditions pose diagnostic challenges. The distinct endoscopic and histological findings, coupled with scintigraphy results, contribute to a comprehensive understanding of this complex case. Differential diagnoses and management considerations are discussed. This case highlights the atypical presentation of Waldmann's disease in adulthood, emphasizing the importance of a multidisciplinary approach for accurate diagnosis and management. Further research is warranted to enhance our understanding of this uncommon disorder and its potential implications for patients with complex medical histories.

13.
Cureus ; 16(8): e66141, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-39233993

RESUMO

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated lymphatic vessels in the gastrointestinal tract, leading to protein-losing enteropathy (PLE). We report the case of a 43-year-old male presenting with refractory PLE, characterized by generalized edema, chronic diarrhea, and significant weight loss over four months. Despite multiple consultations and treatments for presumed inflammatory bowel disease, his symptoms persisted, and his condition worsened. An upper endoscopy was performed, revealing white villi in the duodenum. Histopathological examination of duodenal biopsies confirmed the presence of markedly dilated lymphatic vessels in the lamina propria, consistent with PIL. The patient was managed with a high-protein, low-fat diet supplemented with medium-chain triglycerides (MCTs) and octreotide therapy. This treatment regimen led to significant clinical improvement, including reduced edema, normalization of albumin levels, and resolution of gastrointestinal symptoms. This case underscores the importance of considering PIL in adults with refractory PLE.

14.
Clin Case Rep ; 11(10): e7980, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37808573

RESUMO

Primary intestinal lymphangiectasia is a rare disease that affects children and young adults, causing mainly gastrointestinal disorders that lead to edema and immunologic abnormalities. The majority of patients typically present bilateral lower limb edema, along with pericarditis, pleural effusion, or chylous ascites. The goal of the treatment is to restrict the consumption of long-chain fats and administer a formula that includes protein and medium-chain triglycerides. Here, we report 11-year-old Syrian girl presented to the hospital with 2 years history of diarrhea and generalized edema. Furthermore, investigation showed ascites and pleural effusion. The disease was detected by several biopsies from duodenum that showed dilation of the lymph vessels within the lamina propria without any evidence for inflammation. She was managed by modifying her diet and albumin transfusion, which caused significant improvement in the child's condition.

15.
Intern Med ; 2023 Nov 13.
Artigo em Inglês | MEDLINE | ID: mdl-37952951

RESUMO

A 53-year-old man presented with abdominal symptoms and a fever for 6 months and ascites and lower body edema for 2 months before visiting our clinic. Heart failure, renal failure, inferior vena cava or portal vein obstruction, cirrhosis, and malignancy were suspected, but none were present. We also suspected protein-leakage gastroenteropathy based on the elevated alpha-1 antitrypsin clearance (224 mL/day). Based on the double-balloon endoscopy findings, we diagnosed the patient with primary intestinal lymphangiectasia. Since the patient's ascites were not satisfactorily controlled medically, a Denver peritoneovenous shunt was placed. As a result, the ascites volume was successfully controlled over an extended period.

16.
Cureus ; 15(5): e39085, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37332407

RESUMO

Lymphangiomas are benign malformations of the lymphatic vessels which can be primary or secondary in etiology. Colonic involvement is rare, and the diagnosis is mostly incidental. Sometimes, the initial endoscopic appearance can be misleading. We present a case of colonic lymphangiomatosis presenting with free air under the diaphragm requiring surgical removal of the involved portion of the colon. The diagnosis was confirmed by the pathology of the resected specimen and its correlation with prior clinical information. The patient recovered well with an uneventful postoperative course and follow-up. This case demonstrates a rare complication of colonic lymphangiomatosis prompting definitive treatment by surgical resection.

17.
World J Clin Cases ; 10(18): 6234-6240, 2022 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-35949838

RESUMO

BACKGROUND: Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by abnormally dilated lymphatic structures, resulting in leakage of lymph (rich in protein, lymphocytes, and fat) from the intestinal mucosal and submucosal layers and thus hypoproteinemia, lymphopenia, hypolipidemia, and pleural effusion. CASE SUMMARY: A 19-year-old Chinese male patient complained of recurrent limb convulsions for the last 1 year. Laboratory investigations revealed low levels of calcium and magnesium along with hypoproteinemia and high parathyroid hormone levels, whereas gastroscopy exhibited chronic non-atrophic gastritis and duodenal lymphatic dilatation. Subsequent gastric biopsy showed moderate chronic inflammatory cell infiltration distributed around a small mucosal patch in the descending duodenum followed by lymphatic dilatation in the mucosal lamina propria, which was later diagnosed as PIL. The following appropriate medium-chain triglycerides nutritional support significantly improved the patient's symptoms. CONCLUSION: Since several diseases mimic the clinical symptoms displayed by PIL, like limb convulsions, low calcium and magnesium, and loss of plasma proteins, it is imperative to conduct a detailed analysis to avoid any misdiagnosis while pinpointing the correct clinical diagnosis and simultaneously ruling out other clinical aspects in the reported cases without any past disease history. A careful assessment should always be made to ensure an accurate diagnosis in a timely manner so that the patient can be delivered quality health services for a positive health outcome.

18.
World J Gastroenterol ; 28(22): 2482-2493, 2022 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-35979262

RESUMO

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.


Assuntos
Linfangiectasia Intestinal , Edema/etiologia , Endoscopia Gastrointestinal/efeitos adversos , Humanos , Intestino Delgado/patologia , Linfangiectasia Intestinal/diagnóstico por imagem , Linfangiectasia Intestinal/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X/métodos
19.
Clin Case Rep ; 9(2): 1029-1030, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33598292

RESUMO

This is the first case report comparing endoscopic images of primary intestinal lymphangiectasia (PIL) over 10 years. Regular endoscopic examination is essential in PIL because endoscopic findings do not correlate well with clinical manifestations.

20.
Yonsei Med J ; 62(5): 470-473, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33908219

RESUMO

Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. This procedure is typically reserved for adult patients, although as shown in this case, it can be properly performed in children. His serum albumin level was initially 1.5 g/dL, but elevated to 5.0 g/dL after two sessions of lymphatic embolization. Accordingly, we suggest that embolization could potentially be considered a first-line treatment for focal lesions of primary intestinal IL.


Assuntos
Embolização Terapêutica , Linfangiectasia Intestinal , Enteropatias Perdedoras de Proteínas , Adolescente , Adulto , Biópsia , Criança , Duodeno/diagnóstico por imagem , Humanos , Linfangiectasia Intestinal/diagnóstico por imagem , Linfangiectasia Intestinal/terapia , Masculino , Enteropatias Perdedoras de Proteínas/diagnóstico por imagem , Enteropatias Perdedoras de Proteínas/terapia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA