AUCTORES
Globalize your Research
Case Report | DOI: https://doi.org/10.31579/2692-9392/085
1 Pediatric Hepato-Gastroenterology and Nutrition Department, Rabat Children's Hospital, Faculty of Medicine and Pharmacy, Mohammed V Rabat University, Morocco
2 Anatomopathology Department of the Rabat Children's Hospital, Rabat Children's Hospital, Faculty of Medicine and Pharmacy, Mohammed V University Rabat, Morocco
*Corresponding Author: Khadija Mouaddine, Pediatric Hepato-Gastroenterology and Nutrition Department, Rabat Children's Hospital, Faculty of Medicine and Pharmacy, Mohammed V Rabat University, Morocco.
Citation: Khadija Mouaddine, Mouna Sabib, Laila Benbella, Najat Lamalmi, Nezha Mouane (2022). Waldmann’s Disease Revealed by Chronic Diarrhea and Lymphedema: Case Report. J. Archives of Medical Case Reports and Case Study, 5(2); DOI:10.31579/2692-9392/085
Copyright: © 2022 Khadija Mouaddine, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 25 November 2021 | Accepted: 27 December 2021 | Published: 07 January 2022
Keywords: intestinal lymphangiesctasia; waldmann disease; lymphedema
Intestinal lymphangiesctasia (IL) is a rare disease characterized by the dilation of intestinal lymphatics. It can be classified as primary or secondary depending on the underlying etiology. Usually seen in childhood or adolescence, chronic diarrhea and diffuse edema are the main clinical manifestations of the disease. True lymphoedemas can also be present and affect the lower and upper limbs, they are concomitant with the diagnosis or occur during the course.
The diagnosis is based on the visualization of duodenal lymphangiesctasia. The primitive nature of the disease being affirmed by the elimination of the diseases causing secondary lymphangiectasias. Treatment is based on a strict hypo lipid diet enriched with medium chain triglycerides (MCTs).
We present through this work the observation of a 12-month-old infant who presented with primary intestinal lymphangiesctasia revealed by chronic diarrhea with lymphoedema of the left upper limb.
Intestinal lymphangiesctasia (IL) is a rare and benign disease characterized by focal or diffuse dilation of mucous, sub mucosal and subserosal lymphatics [1]. In addition to being an important cause of protein loss [2], it is frequently associated with extra intestinal lymphatic abnormalities [3].
Depending on the underlying pathology, lymphangiectasia can be classified as primary or secondary disease. The primary form (PIL) probably represents a congenital disorder of the mesenteric lymphatics, as for the secondary form, it may be secondary to diseases such as constrictive pericarditis, lymphoma, sarcoidosis and scleroderma [4].The modification of the diet is the mainstay of the management of this pathology with a variable response.
The objective of our work is to describe the observation of a female infant who presented with primary intestinal lymphangiesctasia revealed by chronic diarrhea with lymphedema of the left upper limb.
Patient and Observation
Our patient is a 12-month-old female infant, from an out-of-consanguineous marriage with healthy siblings, and a well-followed pregnancy. The delivery was vaginally with birth weight = 3400g, exclusive breastfeeding for 02 months then mixed breastfeeding, diversified at the age of 07 months. She has had diarrhea since the age of 6 months at a rate of 6 stools / day. The stools are watery, there is no mucus or blood, and there is progressive abdominal distension and lymphedema of the left upper limb. There are no extra digestive signs, including no respiratory signs and no recurrent infections.
On physical examination, we find a haemodynamically and respiratory stable infant, pale, with a weight of 10kg (M) and a height of 75cm (M). There is no dysmorphia. The abdomen is distended without hepatomegaly or splenomegaly, with the presence of lymphedema of the left hand (Figure 1). No jaundice, no edema, the urine dipstick is negative.
On the biological level: there is a hypoproteinemia at 27g / l with hypo albuminemia at 15g / l. Calcemia is 72mg / l, ferritin is reduced to 8 ng / ml, total cholesterol at 1.37g / l, HDL at 0.27g / l LDL at 0.96 g / l, triglycerides at 0.72 g / l. The liver function tests are normal with ASAT at 47 U / L, ALAT at 18 U / L, GGT at 4 U / L and PAL = 173 .TP at 100%. The blood count reveals a hemoglobin level of 11.1 g / dl with VGM at 73.7 µm3 and CCMH at 31.7%, a leukocyte level at 7060 / µl with polymorphonuclear neutrophils at 4780 / µl and lymphocytes at 1140 / µl and eosinophils at 210 / µl with thrombocytosis at 626000 / µl.
The blood ionogram as well as the renal function are normal, the anti-transglutaminase antibodies are normal with a level of Ig A = 1.25 AU / ml and Ig G = 0.41 AU / ml.
Radiologically: the echo Doppler of the left upper limb as well as the echoceur are normal, the abdominal ultrasound showed the presence of a thickened and agglutinated aspect of the slender loops (7mm) with a slight intraperitoneal effusion.
Jejunal biopsy showed Jejunal mucosa bristling with villi of normal height and lined by a regular columnar epithelium, normosecretant. The glands are well differentiated nomosecretantes. The villus axis is congestive edematous with lesions of lymphangiectasia. The chorion harbors an inflammatory lymphoplasmacytic infiltrate mixed with a few neutrophils (Figure 2).
Secondary causes are ruled out before normal echocardiography, absence of lymphatic obstruction on abdominal ultrasound, normal fecal calprotectin, and negative ASCA and ANCA. The karyotype was normal ruling out Turner syndrome.
The diagnosis of Waldmann's disease was retained in our patient, she received albumin infusions and high protein diet, poor in long-chain fatty acids, enriched in medium-chain fatty acids with supplementation of fat-soluble vitamins and iron.
The evolution was marked by the stabilization of his clinical condition.
Primary intestinal lymphangiectasias or Waldmann's disease were described in 1961 by Waldmann in 18 patients [5], they are due to lymphatic dilations with lymph leakage into the intestinal lumen. It is a disease usually appearing in childhood [6], sometimes with growth retardation in severe forms and of which there are some familial forms [7].
The secondary etiologies of intestinal lymphangiesctasia must be ruled out in order to retain Waldmann disease.
In children, the disease is usually diagnosed before the age of 3, sometimes with very extensive and fatal forms [5, 8]. The main clinical signs are: edema, diarrhea, ascites, asthenia, abdominal pain, nausea, vomiting, weight loss or lack of weight gain, growth retardation. Malabsorption can lead to a deficiency in fat-soluble vitamins (A, D, E, K ), episodes of hypocalcemia sometimes responsible for convulsions [5]. The association with celiac disease has been occasionally reported in children. The degree of severity depends on the anatomical location and the extent of the lymphatic abnormality [9]. While edemas in Waldmann's disease are frequent and associated with hypoproteinemia, lymphoedemas are rarer but can affect the lower and upper limbs. In the course of the disease, they usually occur after gastrointestinal involvement which is symptomatic and prominent [6].
Intestinal lymphangiectasias are recognized during oesogastroduodenal fibroscopy, the suggestive macroscopic appearance corresponds to lymphatic dilations of yellow / brown color (chylomicrons) of variable density and size. Duodenal biopsies confirm the diagnosis with the demonstration of lymphatic dilations, more or less important. The biopsies must be multiple because the lesions can be very localized. There is no villous atrophy or inflammatory infiltrate [10] [11].
The other morphological examinations are of little value. The thoraco-abdomino-pelvic CT scan can show dilation of the slender loops and a thickened aspect of the mucous folds [11]. Indirect laboratory abnormalities are suggestive of LIP: hypoproteinemia, hypo albuminemia, hypogammaglobulinemia (drop in Ig G, A, M) or lymphopenia [12].
The primitive nature of intestinal lymphangiectasias can only be affirmed after having eliminated the secondary causes of digestive lymph loss: malformations of the lymphatic channels, diseases of the lymph nodes (lymphoma, metastases, tuberculosis, filariasis, Whipple's disease), compression of the ducts lymphatics (tumors, retroperitoneal fibrosis, mediastinitis), heart disease with venous pressure, thrombosis of the inferior vena cava, lympho-intestinal fistulas, iatrogenic lesions of the lymphatic system (lymph node excision, radiotherapy) [6].
The treatment is based on a diet low in long chain fatty acids, to limit the pressure in the intestinal lymphatics and prevent their rupture in the digestive lumen. The diet is supplemented with medium chain fatty acids, which are absorbed directly in the portal circulation. Oral supplementation with fat-soluble vitamins (especially D) is essential [12], it is sometimes useful to treat iron deficiency (oral or parenteral). Diuretics may sometimes be necessary in persistent and disabling edema. The treatment generally allows the regression of the laboratory abnormalities but in some cases there may be biological stigmata of lymphatic leakage while the clinical signs may disappear if the diet is strict [13]. Treatment with subcutaneous octreotide has also been reported in a few cases where clinical signs persisted with diet [14] [15]. For lymphedema, it is not sensitive to the fat-free diet but should be treated with standard compression-compression techniques [16].
Primary intestinal lymphangiectasia or Waldmann's disease is a chronic disease requiring a strict, prolonged and restrictive hypo-lipid diet, supplemented with medium-chain triglycerides and fat-soluble vitamins.
In pediatrics, it should be suspected when an infant develops chronic diarrhea: malnutrition, edema, hypoalbuminemia and lymphopenia in the first months of life. The quality of life of these patients is often impaired by the presence of edema and / or lymphedema and frequent asthenia. Early diagnosis and appropriate nutritional treatment corrects the loss of lymphocytes, immunoglobulins, proteins, fats and micronutrients which can lead to repeated infections and, in some cases, death.
Clearly Auctoresonline and particularly Psychology and Mental Health Care Journal is dedicated to improving health care services for individuals and populations. The editorial boards' ability to efficiently recognize and share the global importance of health literacy with a variety of stakeholders. Auctoresonline publishing platform can be used to facilitate of optimal client-based services and should be added to health care professionals' repertoire of evidence-based health care resources.
Journal of Clinical Cardiology and Cardiovascular Intervention The submission and review process was adequate. However I think that the publication total value should have been enlightened in early fases. Thank you for all.
Journal of Women Health Care and Issues By the present mail, I want to say thank to you and tour colleagues for facilitating my published article. Specially thank you for the peer review process, support from the editorial office. I appreciate positively the quality of your journal.
Journal of Clinical Research and Reports I would be very delighted to submit my testimonial regarding the reviewer board and the editorial office. The reviewer board were accurate and helpful regarding any modifications for my manuscript. And the editorial office were very helpful and supportive in contacting and monitoring with any update and offering help. It was my pleasure to contribute with your promising Journal and I am looking forward for more collaboration.
We would like to thank the Journal of Thoracic Disease and Cardiothoracic Surgery because of the services they provided us for our articles. The peer-review process was done in a very excellent time manner, and the opinions of the reviewers helped us to improve our manuscript further. The editorial office had an outstanding correspondence with us and guided us in many ways. During a hard time of the pandemic that is affecting every one of us tremendously, the editorial office helped us make everything easier for publishing scientific work. Hope for a more scientific relationship with your Journal.
The peer-review process which consisted high quality queries on the paper. I did answer six reviewers’ questions and comments before the paper was accepted. The support from the editorial office is excellent.
Journal of Neuroscience and Neurological Surgery. I had the experience of publishing a research article recently. The whole process was simple from submission to publication. The reviewers made specific and valuable recommendations and corrections that improved the quality of my publication. I strongly recommend this Journal.
Dr. Katarzyna Byczkowska My testimonial covering: "The peer review process is quick and effective. The support from the editorial office is very professional and friendly. Quality of the Clinical Cardiology and Cardiovascular Interventions is scientific and publishes ground-breaking research on cardiology that is useful for other professionals in the field.
Thank you most sincerely, with regard to the support you have given in relation to the reviewing process and the processing of my article entitled "Large Cell Neuroendocrine Carcinoma of The Prostate Gland: A Review and Update" for publication in your esteemed Journal, Journal of Cancer Research and Cellular Therapeutics". The editorial team has been very supportive.
Testimony of Journal of Clinical Otorhinolaryngology: work with your Reviews has been a educational and constructive experience. The editorial office were very helpful and supportive. It was a pleasure to contribute to your Journal.
Dr. Bernard Terkimbi Utoo, I am happy to publish my scientific work in Journal of Women Health Care and Issues (JWHCI). The manuscript submission was seamless and peer review process was top notch. I was amazed that 4 reviewers worked on the manuscript which made it a highly technical, standard and excellent quality paper. I appreciate the format and consideration for the APC as well as the speed of publication. It is my pleasure to continue with this scientific relationship with the esteem JWHCI.
This is an acknowledgment for peer reviewers, editorial board of Journal of Clinical Research and Reports. They show a lot of consideration for us as publishers for our research article “Evaluation of the different factors associated with side effects of COVID-19 vaccination on medical students, Mutah university, Al-Karak, Jordan”, in a very professional and easy way. This journal is one of outstanding medical journal.
Dear Hao Jiang, to Journal of Nutrition and Food Processing We greatly appreciate the efficient, professional and rapid processing of our paper by your team. If there is anything else we should do, please do not hesitate to let us know. On behalf of my co-authors, we would like to express our great appreciation to editor and reviewers.
As an author who has recently published in the journal "Brain and Neurological Disorders". I am delighted to provide a testimonial on the peer review process, editorial office support, and the overall quality of the journal. The peer review process at Brain and Neurological Disorders is rigorous and meticulous, ensuring that only high-quality, evidence-based research is published. The reviewers are experts in their fields, and their comments and suggestions were constructive and helped improve the quality of my manuscript. The review process was timely and efficient, with clear communication from the editorial office at each stage. The support from the editorial office was exceptional throughout the entire process. The editorial staff was responsive, professional, and always willing to help. They provided valuable guidance on formatting, structure, and ethical considerations, making the submission process seamless. Moreover, they kept me informed about the status of my manuscript and provided timely updates, which made the process less stressful. The journal Brain and Neurological Disorders is of the highest quality, with a strong focus on publishing cutting-edge research in the field of neurology. The articles published in this journal are well-researched, rigorously peer-reviewed, and written by experts in the field. The journal maintains high standards, ensuring that readers are provided with the most up-to-date and reliable information on brain and neurological disorders. In conclusion, I had a wonderful experience publishing in Brain and Neurological Disorders. The peer review process was thorough, the editorial office provided exceptional support, and the journal's quality is second to none. I would highly recommend this journal to any researcher working in the field of neurology and brain disorders.
Dear Agrippa Hilda, Journal of Neuroscience and Neurological Surgery, Editorial Coordinator, I trust this message finds you well. I want to extend my appreciation for considering my article for publication in your esteemed journal. I am pleased to provide a testimonial regarding the peer review process and the support received from your editorial office. The peer review process for my paper was carried out in a highly professional and thorough manner. The feedback and comments provided by the authors were constructive and very useful in improving the quality of the manuscript. This rigorous assessment process undoubtedly contributes to the high standards maintained by your journal.
International Journal of Clinical Case Reports and Reviews. I strongly recommend to consider submitting your work to this high-quality journal. The support and availability of the Editorial staff is outstanding and the review process was both efficient and rigorous.
Thank you very much for publishing my Research Article titled “Comparing Treatment Outcome Of Allergic Rhinitis Patients After Using Fluticasone Nasal Spray And Nasal Douching" in the Journal of Clinical Otorhinolaryngology. As Medical Professionals we are immensely benefited from study of various informative Articles and Papers published in this high quality Journal. I look forward to enriching my knowledge by regular study of the Journal and contribute my future work in the field of ENT through the Journal for use by the medical fraternity. The support from the Editorial office was excellent and very prompt. I also welcome the comments received from the readers of my Research Article.
Dear Erica Kelsey, Editorial Coordinator of Cancer Research and Cellular Therapeutics Our team is very satisfied with the processing of our paper by your journal. That was fast, efficient, rigorous, but without unnecessary complications. We appreciated the very short time between the submission of the paper and its publication on line on your site.
I am very glad to say that the peer review process is very successful and fast and support from the Editorial Office. Therefore, I would like to continue our scientific relationship for a long time. And I especially thank you for your kindly attention towards my article. Have a good day!
"We recently published an article entitled “Influence of beta-Cyclodextrins upon the Degradation of Carbofuran Derivatives under Alkaline Conditions" in the Journal of “Pesticides and Biofertilizers” to show that the cyclodextrins protect the carbamates increasing their half-life time in the presence of basic conditions This will be very helpful to understand carbofuran behaviour in the analytical, agro-environmental and food areas. We greatly appreciated the interaction with the editor and the editorial team; we were particularly well accompanied during the course of the revision process, since all various steps towards publication were short and without delay".
I would like to express my gratitude towards you process of article review and submission. I found this to be very fair and expedient. Your follow up has been excellent. I have many publications in national and international journal and your process has been one of the best so far. Keep up the great work.
We are grateful for this opportunity to provide a glowing recommendation to the Journal of Psychiatry and Psychotherapy. We found that the editorial team were very supportive, helpful, kept us abreast of timelines and over all very professional in nature. The peer review process was rigorous, efficient and constructive that really enhanced our article submission. The experience with this journal remains one of our best ever and we look forward to providing future submissions in the near future.
I am very pleased to serve as EBM of the journal, I hope many years of my experience in stem cells can help the journal from one way or another. As we know, stem cells hold great potential for regenerative medicine, which are mostly used to promote the repair response of diseased, dysfunctional or injured tissue using stem cells or their derivatives. I think Stem Cell Research and Therapeutics International is a great platform to publish and share the understanding towards the biology and translational or clinical application of stem cells.
I would like to give my testimony in the support I have got by the peer review process and to support the editorial office where they were of asset to support young author like me to be encouraged to publish their work in your respected journal and globalize and share knowledge across the globe. I really give my great gratitude to your journal and the peer review including the editorial office.
I am delighted to publish our manuscript entitled "A Perspective on Cocaine Induced Stroke - Its Mechanisms and Management" in the Journal of Neuroscience and Neurological Surgery. The peer review process, support from the editorial office, and quality of the journal are excellent. The manuscripts published are of high quality and of excellent scientific value. I recommend this journal very much to colleagues.
Dr.Tania Muñoz, My experience as researcher and author of a review article in The Journal Clinical Cardiology and Interventions has been very enriching and stimulating. The editorial team is excellent, performs its work with absolute responsibility and delivery. They are proactive, dynamic and receptive to all proposals. Supporting at all times the vast universe of authors who choose them as an option for publication. The team of review specialists, members of the editorial board, are brilliant professionals, with remarkable performance in medical research and scientific methodology. Together they form a frontline team that consolidates the JCCI as a magnificent option for the publication and review of high-level medical articles and broad collective interest. I am honored to be able to share my review article and open to receive all your comments.
“The peer review process of JPMHC is quick and effective. Authors are benefited by good and professional reviewers with huge experience in the field of psychology and mental health. The support from the editorial office is very professional. People to contact to are friendly and happy to help and assist any query authors might have. Quality of the Journal is scientific and publishes ground-breaking research on mental health that is useful for other professionals in the field”.
Dear editorial department: On behalf of our team, I hereby certify the reliability and superiority of the International Journal of Clinical Case Reports and Reviews in the peer review process, editorial support, and journal quality. Firstly, the peer review process of the International Journal of Clinical Case Reports and Reviews is rigorous, fair, transparent, fast, and of high quality. The editorial department invites experts from relevant fields as anonymous reviewers to review all submitted manuscripts. These experts have rich academic backgrounds and experience, and can accurately evaluate the academic quality, originality, and suitability of manuscripts. The editorial department is committed to ensuring the rigor of the peer review process, while also making every effort to ensure a fast review cycle to meet the needs of authors and the academic community. Secondly, the editorial team of the International Journal of Clinical Case Reports and Reviews is composed of a group of senior scholars and professionals with rich experience and professional knowledge in related fields. The editorial department is committed to assisting authors in improving their manuscripts, ensuring their academic accuracy, clarity, and completeness. Editors actively collaborate with authors, providing useful suggestions and feedback to promote the improvement and development of the manuscript. We believe that the support of the editorial department is one of the key factors in ensuring the quality of the journal. Finally, the International Journal of Clinical Case Reports and Reviews is renowned for its high- quality articles and strict academic standards. The editorial department is committed to publishing innovative and academically valuable research results to promote the development and progress of related fields. The International Journal of Clinical Case Reports and Reviews is reasonably priced and ensures excellent service and quality ratio, allowing authors to obtain high-level academic publishing opportunities in an affordable manner. I hereby solemnly declare that the International Journal of Clinical Case Reports and Reviews has a high level of credibility and superiority in terms of peer review process, editorial support, reasonable fees, and journal quality. Sincerely, Rui Tao.
Clinical Cardiology and Cardiovascular Interventions I testity the covering of the peer review process, support from the editorial office, and quality of the journal.
Clinical Cardiology and Cardiovascular Interventions, we deeply appreciate the interest shown in our work and its publication. It has been a true pleasure to collaborate with you. The peer review process, as well as the support provided by the editorial office, have been exceptional, and the quality of the journal is very high, which was a determining factor in our decision to publish with you.
The peer reviewers process is quick and effective, the supports from editorial office is excellent, the quality of journal is high. I would like to collabroate with Internatioanl journal of Clinical Case Reports and Reviews journal clinically in the future time.
Clinical Cardiology and Cardiovascular Interventions, I would like to express my sincerest gratitude for the trust placed in our team for the publication in your journal. It has been a true pleasure to collaborate with you on this project. I am pleased to inform you that both the peer review process and the attention from the editorial coordination have been excellent. Your team has worked with dedication and professionalism to ensure that your publication meets the highest standards of quality. We are confident that this collaboration will result in mutual success, and we are eager to see the fruits of this shared effort.
