Congenital intestinal lymphangiectasia

Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

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Diagnosis and Individualized Nutritional Management of Primary Intestinal Lymphangiectasia in Children: A Clinical Conundrum

10.21203/rs.3.rs-552541/v1 ◽

2021 ◽

Author(s):

Yang Niu ◽

Qingqing Wu ◽

Ying Wang ◽

Lina Lu ◽

Wei Cai ◽

...

Keyword(s):

Clinical Symptoms ◽

Clinical Manifestations ◽

Lymphatic Vessels ◽

High Protein Diet ◽

Diet Therapy ◽

Abdominal Distention ◽

Intestinal Lymphangiectasia ◽

School Of Medicine ◽

Hospital School

Abstract Background: Primary Intestinal Lymphangiectasia (PIL) is a rare congenital and digestive disease, which could present through a broad spectrum of clinical manifestations, diagnostic and treatment management. The aim of this study was to introduce the diagnosis and nutrition treatment of children with IL through the twelve years of experience. Methods: The patients diagnosed with PIL admitted to the Department of Gastroenterology and Nutrition in Xinhua Hospital from June, 2006 to September, 2017 were included in the study. Basic information and laboratory results were collected from the medical records. Results: Ten patients were found to have PIL. The mean age was 66 months at the time of diagnosis and 11 months at onset. The main clinical manifestations were diarrhea, edemas and abdominal distention. Marked dilatation of the intestinal lymphatic vessels was the characteristic of the endoscopic. All the patients presented with hypoproteinemia and hypoimmunoglobulinia. 6 of them were treated with parenteral nutrition, and 9 of them were treated with a low-LCT (long-chain triglycerides), high-protein diet supplemented with MCT(medium-chain triglycerides).The clinical symptoms of the patients have improved after the MCT diet therapy. Conclusion: PIL should be considered first when there are clinical manifestations of chronic diarrhea, edema and abdominal distention, and biochemical results indicated the hypoproteinemia and hypoimmunoglobulinia, and the general treatment is invalid. Gastroscopy and E-colonoscopy with biopsies are the preferred method of diagnosis. MCT diet is the cornerstone and longtime medical treatment, which can improve the nutritional status and promote the survival quality of patients with IL.Trial registration: This study was reviewed and approved by the Research Ethics Committee of Xinhua Hospital, School of Medicine, Shanghai Jiao Tong University.

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Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia

Case Reports in Medicine ◽

10.1155/2020/7870154 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Fernando Naranjo-Saltos ◽

Alejandro Hallo ◽

Carlos Hallo ◽

Andres Mayancela ◽

Alejandra Rojas

Keyword(s):

Opportunistic Infections ◽

Lymphatic Vessels ◽

Immunocompetent Patient ◽

Intestinal Lumen ◽

Histopathological Study ◽

Intestinal Lymphangiectasia ◽

Protein Losing Enteropathy ◽

Unusual Association ◽

Intestinal Involvement ◽

Cellular Components

Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. An intestinal histopathological study revealed the unusual association of lymphangiectasia and intestinal cryptococcosis. Although cryptococcal infection is common in immunocompromised patients, intestinal involvement is rarely reported. We found no reports on the association of intestinal cryptococcosis in patients with lymphangiectasia. This case report is the first to describe intestinal cryptococcosis associated with intestinal lymphangiectasia.

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Lymphatics in the Alimentary Tract of Children in Health and Disease: Study on Mucosal Biopsies Using the Monoclonal Antibody D2-40

Pediatric and Developmental Pathology ◽

10.1007/s10024-005-0023-x ◽

2005 ◽

Vol 8 (5) ◽

pp. 541-549 ◽

Cited By ~ 14

Author(s):

Yiping Zeng ◽

Fenghua Wang ◽

Elizabeth D. Williams ◽

Chung Wo Chow

Keyword(s):

Clinical Features ◽

Alimentary Tract ◽

Lymphatic Vessels ◽

Specific Method ◽

Intestinal Lymphangiectasia ◽

Histologic Diagnosis ◽

Protein Losing Enteropathy ◽

Negative Finding ◽

Young Infants ◽

Disease Study

Primary intestinal lymphangiectasia and intestinal lymphatic hypoplasia are 2 causes of protein-losing enteropathy in children and share many common clinical features. For the diagnosis of lymphatic hypoplasia on endoscopic biopsies of the intestine, i.e., based on a negative finding in a small specimen, a very sensitive and specific method for identifying lymphatics is essential. In the present study, lymphatic vessels were labelled using D2-40 immunostaining in mucosal biopsy specimens of the alimentary tract of children in whom no histologic abnormality was noted and of those who had different relatively common pediatric conditions, including inflammatory and neoplastic diseases. Using this method, lymphatic vessels were well visualized even in young infants and not destroyed by diseases. The presence of the muscularis mucosae in specimens was important for adequate assessment. In the duodenum and esophagus, lymphatics were observed in every single section; in the stomach, ileum, and colon, they were less regular and several sections were sometimes required. The extreme sensitivity of this method for demonstrating lymphatic vessels in the duodenum makes it ideal for the histologic diagnosis of intestinal lymphatic hypoplasia. In 4 patients who were considered to have this diagnosis based on clinical features, full-thickness intestinal biopsies and electron microscopy, D2-40 immunostaining confirmed the absence or marked paucity of lymphatics.

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Primary lymphangiectasia of the gastrointestinal tract

Terapevticheskii arkhiv ◽

10.26442/terarkh201890396-98 ◽

2018 ◽

Vol 90 (3) ◽

pp. 96-98

Author(s):

E M Esedov ◽

A S Abasova ◽

F D Akhmedova ◽

L N Musaeva

Keyword(s):

Trace Elements ◽

Gastrointestinal Tract ◽

Lymphatic Vessels ◽

Intestinal Wall ◽

Symptomatic Therapy ◽

Intestinal Lymphangiectasia ◽

Medium Chain Triglycerides ◽

Medicinal Foods ◽

Instrumental Methods ◽

Test Treatment

Intestinal lymphangiectasia is a very rare pathology, characterized by the presence of enlarged lymphatic vessels in all layers of the intestinal wall and in the mesentery. As a result, "lymphatic lakes" are formed, through which lymph exudates into the lumen of the intestine. The main manifestation is hypoproteinemic edema. Diagnosis of the disease is based on laboratory-instrumental methods of investigation, including by determining in the stool α-1 antitrypsin as a screening test. Treatment includes: a diet rich in calcium, trace elements, be sure to use medicinal foods enriched with medium chain triglycerides, substitution and symptomatic therapy.

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Superior Orbital Fissure Syndrome due to Sinusitis: Report of Two Cases

American Journal of Rhinology ◽

10.1177/194589240501900417 ◽

2005 ◽

Vol 19 (4) ◽

pp. 417-420 ◽

Cited By ~ 2

Author(s):

Fabio de Rezende Pinna ◽

Daniel L. Dutra ◽

Maura C. Neves ◽

Fabrizio Ricci Romano ◽

Richard L. Voegels ◽

...

Keyword(s):

Early Recognition ◽

Complete Recovery ◽

Ocular Motility ◽

Intravenous Antibiotic ◽

Superior Orbital Fissure ◽

Adequate Treatment ◽

Superior Orbital Fissure Syndrome ◽

Brain Involvement ◽

The Brain ◽

Anatomic Region

Background The potential morbidity and mortality of sphenoid sinus infectious processes are related to their proximity to vital structures within the orbit, such as the cavernous sinus and the brain. Involvement of the posterior orbit can result in superior orbital fissure syndrome, a rare entity affecting structures that cross this anatomic region. Early recognition of this syndrome is mandatory. Delays in adequate treatment may compromise the patient's prognosis. Methods We present two cases of incomplete superior orbital fissure syndrome due to infectious processes of the posterior ethmoid and sphenoid sinuses. Conclusions In our experience, endoscopic decompression of the lamina papyracea shows complete recovery of extrinsic ocular motility in these patients and should be combined with intravenous antibiotic therapy.

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SPECIAL ARTICLE

PEDIATRICS ◽

10.1542/peds.15.5.642 ◽

1955 ◽

Vol 15 (5) ◽

pp. 642-646

Keyword(s):

General Population ◽

Rheumatic Fever ◽

Streptococcal Infection ◽

Acute Otitis Media ◽

Clinical Manifestations ◽

Diagnostic Errors ◽

White Blood Count ◽

Streptococcal Infections ◽

Laboratory Findings ◽

Adequate Treatment

RHEUMAT1C fever is a recurrent disease which in most instances can be prevented. Since both the initial and recurrent attacks of the disease are precipitated by infections with beta hemolytic streptococci, prevention of rheumatic fever and rheumatic heart disease depends upon the control of streptococcal infections. This may be accomplished by (1) early and adequate treatment of streptococcal infections in all individuals and (2) prevention of streptococcal infections in rheumatic subjects. TREATMENT OF STREPTOCOCCAL INFECTIONS IN THE GENERAL POPULATION In the general population about 3 per cent of untreated streptococcal infections are followed by rheumatic fever. Adequate and early penicillin treatment, however, will eliminate streptococci from the throat and prevent most attacks of rheumatic fever. Diagnosis of Streptococcal Infection In many instances streptococcal infections can be recognized by their clinical manifestations. In some patients, however, it is difficult or impossible to determine the streptococcal nature of a respiratory infection without obtaining throat cultures. The following section on diagnosis has been included in order to reduce diagnostic errors and to assist physicians in avoiding unnecessary therapy. The accurate recognition of individual streptococcal infections, their adequate treatment and the control of epidemics in the community presently offer the best means of preventing initial and recurrent rheumatic fever. Common Symptoms Sore Throat—sudden onset, pain on swallowing. Headache—common. Fever—variable, but generally from 101° to 104°F. Abdominal Pain—common, especially in children; less common in adults. Nausea and Vomiting—common, especially in children. Common Signs Red Throat. Exudate—usually present. Glands—swollen, tender lymph nodes at angle of jaw. Rash—scarlatiniform. Acute Otitis Media and Acute Sinusitis —frequently due to the streptococcus. In the absence of the common symptoms and signs occurrence of any of the following symptoms is usually not associated with a streptococcal infection : simple coryza; hoarseness; cough. Laboratory Findings White Blood Count—generally over 12,000. Throat Culture—positive culture for hemolytic streptococci is almost always diagnostic.

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Current Diagnosis and Management of Cervical Spondylotic Myelopathy

Global Spine Journal ◽

10.1177/2192568217699208 ◽

2017 ◽

Vol 7 (6) ◽

pp. 572-586 ◽

Cited By ~ 45

Author(s):

Joshua Bakhsheshian ◽

Vivek A. Mehta ◽

John C. Liu

Keyword(s):

Natural History ◽

Cervical Spondylotic Myelopathy ◽

Early Recognition ◽

Clinical Manifestations ◽

High Quality ◽

Diagnosis And Management ◽

Imaging Characteristics ◽

Cervical Sagittal Alignment ◽

Long Term Follow Up ◽

The Cost

Study Design: Review. Objectives: Cervical spondylotic myelopathy (CSM) is a major cause of disability, particular in elderly patients. Awareness and understanding of CSM is imperative to facilitate early diagnosis and management. This review article addresses CSM with regard to its epidemiology, anatomical considerations, pathophysiology, clinical manifestations, imaging characteristics, treatment approaches and outcomes, and the cost-effectiveness of surgical options. Methods: The authors performed an extensive review of the peer-reviewed literature addressing the aforementioned objectives. Results: The clinical presentation and natural history of CSM is variable, alternating between quiescent and insidious to stepwise decline or rapid neurological deterioration. For mild CSM, conservative options could be employed with careful observation. However, surgical intervention has shown to be superior for moderate to severe CSM. The success of operative or conservative management of CSM is multifactorial and high-quality studies are lacking. The optimal surgical approach is still under debate, and can vary depending on the number of levels involved, location of the pathology and baseline cervical sagittal alignment. Conclusions: Early recognition and treatment of CSM, before the onset of spinal cord damage, is essential for optimal outcomes. The goal of surgery is to decompress the cord with expansion of the spinal canal, while restoring cervical lordosis, and stabilizing when the risk of cervical kyphosis is high. Further high-quality randomized clinical studies with long-term follow up are still needed to further define the natural history and help predict the ideal surgical strategy.

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Intracranial Haemorrhage as a Fatal Complication of Evans Syndrome: A Case Report

Nepal Medical Journal ◽

10.37080/nmj.76 ◽

2019 ◽

Vol 2 (2) ◽

pp. 66-69

Author(s):

Olita Shilpakar ◽

Bibek Rajbhandari ◽

Bipin Karki ◽

Umesh Bogati

Keyword(s):

Intracranial Haemorrhage ◽

Early Recognition ◽

Clinical Manifestations ◽

Health Care Facilities ◽

Evans Syndrome ◽

Fatal Complication ◽

Hematologic Disorder ◽

Immune Mediated ◽

Life Threatening ◽

Immune Neutropenia

Evans syndrome is a rare hematologic disorder characterized by the presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA), immune-mediated thrombocytopenia and/or immune neutropenia without any known underlying etiology. Spontaneous intracranial hemorrhage is a rare and life-threatening complication in patients with Evans syndrome and very few cases have been reported to date. We report a case of a thirty-two- year-old female with intracranial haemorrhage with underlying Evans syndrome who presented with the clinical manifestations of headache, vomiting and altered sensorium and succumbed to the fatal complication despite resuscitative measures. This also emphasizes the importance of early recognition of symptoms and immediate presentation to health care facilities for aggressive management of the patient.

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Clinical, Aetiologic, And Evolution-Related Aspects Of Pleurisy In Children

ARS Medica Tomitana ◽

10.1515/arsm-2015-0048 ◽

2015 ◽

Vol 21 (4) ◽

pp. 212-216

Author(s):

Frecus Corina ◽

Balasa Adriana ◽

Ungureanu Adina ◽

Mihai Larisia ◽

Cuzic Viviana ◽

...

Keyword(s):

Environmental Factors ◽

Clinical Manifestations ◽

Clinical Aspects ◽

Age Group ◽

Adequate Treatment ◽

Radiologic Imaging ◽

Specific Pathogen ◽

Laboratory Investigations ◽

Group Distribution ◽

Clinical Emergency

Abstract Objectives: Establishing the frequency of pleurisy from the total number of admissions in the Paediatrics Department, as well as the frequency of pleurisy from the total number of respiratory ailments that required hospitalization; evaluating age group distribution and determining the influence of environmental factors; describing clinical manifestations, laboratory, radiologic, and bacteriological investigations in patients with pleurisy; quantifying clinical manifestations and investigations so as to establish an appropriate therapeutic approach; identifying clinical aspects that indicate a favourable/unfavourable evolution; analyzing the evolution of cases after treatment as revealed by radiologic imaging. Method: Retrospective study on 47 patients diagnosed with pleurisy and admitted in the Paediatrics Department of Constanta Clinical Emergency Hospital, over a span of 3 years (2011-2013), based on data collected from observation sheets. Results: A downward trend in what regards the frequency of pleurisy can be observed from 2011 to 2013. Rural provenience and other environmental factors continue to play an important role. Clinical manifestations tend to be more significant for younger patients and they are directly related to the specific pathogen identified during laboratory investigations. Conclusion: Following an early diagnosis and a subsequent adequate treatment, evolution tends to be favourable in most cases, although certain post-hospitalization measures still need to be implemented in order to ensure full recovery and restitution ad integrum.

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CARDIAL MANIFESTATIONS OF NON-DIFFERENTIATED CONNECTIVE TISSUE DYSPLASIA AND THEIR INFLUENCE ON PREGNANCY

Scientific digest of association of obstetricians and gynecologists of Ukraine ◽

10.35278/2664-0767.1(47).2021.242663 ◽

2021 ◽

pp. 14-22

Author(s):

V. Yu. DOBRIANSKA ◽

S. M. HERYAK ◽

L. M. MALANCHUK ◽

M. I. SHVED ◽

I. V. KORDA

Keyword(s):

Mitral Valve ◽

Connective Tissue ◽

Ventricular Arrhythmia ◽

Pregnant Women ◽

Mitral Valve Prolapse ◽

Clinical Signs ◽

Clinical Manifestations ◽

Adequate Treatment ◽

Pregnancy And Childbirth ◽

Connective Tissue Dysplasia

Among the visceral manifestations of NDST in pregnant women most often diagnosed mitral valve prolapse (20-25%) that accompanied by more cardiovascular and obstetric complications during pregnancy. It demonstrates the high clinical significance of the problem of connective tissue dysplasia with mitral valve prolaps for pregnancy and requires adequate treatment programs for prevention of complications and management of pregnant women with connective tissue dysplasia. Aim. Determination of the frequency of pregnancy pathology in women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia. Materials and methods. 138 pregnant women with MVP and concomitant signs of NDST and 54 healthy pregnant women were selected for analysis. Clinical manifestations of NDST, different variants of arrhythmias and the total number of complications of pregnancy and childbirth were evaluated. Results. In pregnant women with clinical signs of NDST and MVP complicated by extrasystolic arrhythmia, cases of frequent sinus extrasystole were significantly more often compared to frequent ventricular arrhythmia (47.8% vs. 18.1%, p<0.001) and cases of combination of frequent sinus extrasystole and ventricular arrhythmia (13.3 % vs. 1.5%, p <0.05). They significantly more often identified both symptoms of arrhythmological nature and symptoms that indicated a violation of autonomic status. The presence of NDST syndrome is more often accompanied by the development of complications of pregnancy and childbirth. These pregnant women have genetic and phenotypic risk factors for the development of pathological pregnancy and childbirth, birth trauma, disability of mother and newborn, which justifies such patients in a separate risk group for individualized programs of the prevention and treatment of visceral (cardiac) manifestations of NDST and possible complications of pregnancy and childbirth. Conclusions. 3.1% of pregnant women are diagnosed with phenotypic signs (stigma) of undifferentiated connective tissue dysplasia, and the most common visceral cardiac manifestation is mitral valve prolapse. The presence of mitral valve prolapse and extrasystolic arrhythmia in pregnant women with NDST is accompanied by significantly more frequent development of pregnancy and childbirth complications in these patients.

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