scholarly journals Waldmann’s Disease Revealed by Chronic Diarrhea and Lymphedema: Case Report

2022 ◽  
Vol 5 (2) ◽  
pp. 01-04
Author(s):  
Khadija Mouaddine ◽  
Mouna Sabib ◽  
Laila Benbella ◽  
Najat Lamalmi ◽  
Nezha Mouane
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Upper Limb ◽  
Chronic Diarrhea ◽  
Clinical Manifestations ◽  
Upper Limbs ◽  
Medium Chain Triglycerides ◽  
Medium Chain ◽  
Intestinal Lymphatics ◽  
Lipid Diet

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.

scholarly journals Intestinal lymphangiectasia: case report

2019 ◽  
Vol 6 (5) ◽  
pp. 1678
Author(s):  
Reham Mohmmad Aljohnei ◽  
Hawazen Yousef Abdullah Alani
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Present Report ◽  
Abdominal Distention ◽  
Intestinal Lymphangiectasia ◽  
Peripheral Edema ◽  
Long Duration ◽  
White Spots ◽  
Intestinal Lymphatics ◽  
History Of

Primary intestinal lymphangiectasia (PIL) was first described by Waldmann et al, in 1961. PIL is a rare disease with several hundred reported cases. It is rarely reported in adults because it is presumably a congenital disorder and when present in adults it typically produces a long duration of manifestation such as diarrhea, abdominal distention from ascites, and peripheral edema. This disorder is characterized by markedly dilated intestinal lymphatics, hypoproteinemia, generalized edema, lymphocytopenia hypogammaglobinemia, and immunologic anomalies. The loss of protein into the from dilated intestinal lymphatics leads to the development of hypoproteinemia in these patients and its demonstration is important in the diagnosis of intestinal lymphangiectasia. The disease can be secondary to congenital, secondary or idiopathic defects in the formation of the lymphatic ducts. In the present report, we describe a case of 15 years old female presented to our hospital with history of generalized edema, bilateral hand spasm, and diarrhea. Endoscopy of the patient revealed White spots (dilated lacteals), white nodules, and submucosal elevations were observed. Changes suggestive of the disease includes White villi and/or spots (dilated lacteals), white nodules, and submucosal elevations are observed. Xanthomata’s plaques are often visualized, there are no specific treatments for patients with PIL. treatment of patients with primary intestinal lymphangiectasia involves control of symptoms with the use of dietary, pharmaceutical, and behavioral modifications.

scholarly journals Benign monomelic amyotrophy with proximal upper limb involvement: case report

2007 ◽  
Vol 65 (2b) ◽  
pp. 524-527 ◽  
Author(s):  
Marco Antonio Orsini Neves ◽  
Marcos R.G. de Freitas ◽  
Mariana Pimentel de Mello ◽  
Carlos Henrique Dumard ◽  
Gabriel R. de Freitas ◽  
...  
Keyword(s):  
Case Report ◽  
Upper Limb ◽  
Lower Limb ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Insidious Onset ◽  
Rare Location ◽  
Muscle Groups ◽  
Clinical Stabilization ◽  
The Right

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .

scholarly journals Facilitating Upper Limb Function using Assistive Technology in a Neurological Patient with Bilateral Upper Limb Impairment: A Case Report

2020 ◽  
Author(s):  
Shelley Fulton ◽  
Laura Wilkinson ◽  
Kathy Stiller
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injury ◽  
Case Report ◽  
Assistive Technology ◽  
Upper Limb ◽  
Daily Living ◽  
Assistive Device ◽  
Outpatient Rehabilitation ◽  
Upper Limbs ◽  
Cord Injury

Purpose: A combination of a stroke and spinal cord injury adversely affecting both upper limbs is an unusual combination for a patient presenting for outpatient rehabilitation services. Although the management of these conditions in isolation is well documented, there is limited literature regarding rehabilitation for these conditions in combination, particularly the use of assistive technology in this setting. Methods: A case report is presented of a 53-year-old male referred for outpatient rehabilitation following a left-sided stroke, with resultant dense right sided hemiplegia. A pre-existing spinal cord injury had affected his left upper limb such that he had marked proximal weakness. This combination of impairments meant he was unable to perform even basic activities of daily living involving the upper limbs. A therapy program, led by an occupational therapist with support and input from the multi-disciplinary team, included the use of an assistive device (a mobile arm support) to facilitate functional upper limb activities. This greatly improved his ability to do upper limb functional activities. Conclusion: The use of an assistive device enabled the patient to engage in meaningful activities of daily living involving the upper limbs.

scholarly journals Hemorrhagic thrombocytopathy with defective signal transduction CalDAG-GEFI

2021 ◽  
Vol 20 (3) ◽  
pp. 126-131
Author(s):  
A. O. Koposova ◽  
D. V. Fedorova ◽  
A. V. Pshonkin ◽  
A. V. Poletaev ◽  
E. A. Seregina ◽  
...  
Keyword(s):  
Signal Transduction ◽  
Case Report ◽  
Rare Disease ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
The World ◽  
The Moment

Hemorrhagic thrombocytopathy with defective signal transduction CalDAG-GEFI is a rare disease associated with a mutation in the RASGRP2 gene. At the moment, this disease is described in 10 person in the world. We present clinical case report of this pathology of a 9-year-old child. We also offer a review of the available literature about pathogenetic features, clinical manifestations and prevalence of this rare disease. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications.

scholarly journals Keratosis Lichenoides Chronica – a Case Report and Literature Review

2014 ◽  
Vol 6 (3) ◽  
pp. 120-137
Author(s):  
Mirjana Paravina ◽  
Predrag Cvetanović ◽  
Miloš Kostov ◽  
Slađana Živković ◽  
Ivana Dimovski ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Literature Review ◽  
Rare Disease ◽  
Clinical Picture ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Poor Response ◽  
Unknown Etiology ◽  
Distinct Entity

Abstract Keratosis lichenoides chronica represents a distinct entity, a rare disease of unknown etiology and pathogenesis, with clinical manifestations which, although typical, require extensive differential diagnosis. The course of the disease is chronic, progressive, and it is resistant to various treatment options, so despite variations in the clinical picture it is really easier to diagnose than to treat. This is a case report of a male patient in whom the diagnosis of keratosis lichenoides chronica was based on typical clinical picture, repeated biopsies and histopathological findings, course of the disease and poor response to any therapy.

scholarly journals Letterer Siwe Disease (LSD): A Case Report

2018 ◽  
Vol 13 (3) ◽  
pp. 207
Author(s):  
Suad H H ◽  
Mona Mohamed Elamin ◽  
Gad Allah Modawe ◽  
Khalid AbdElmohsin Awad Elseed
Keyword(s):  
Dendritic Cells ◽  
Case Report ◽  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
The Body ◽  
System Disease

 Background: Letterer Siwe Disease (LSD) is one of variants of langerhans cell histiocytosis (LCH) which is considered as a rare disease that affects many systems in the body, it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve other organs as well or appear as a multi-system disease leading to different clinical manifestations and eventually death.

scholarly journals Ollier disease: multiple enchondromatosis: case report and review of literature

2021 ◽  
Vol 9 (6) ◽  
pp. 1770
Author(s):  
Hirosi Sashida Mendez ◽  
Maria de los Angeles Mendoza Velez ◽  
Luisa Hurtado Diaz ◽  
Jorge Rojas Ortiz ◽  
Edgardo Araiza Gomez
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
World Literature ◽  
Clinical Manifestations ◽  
Review Of Literature ◽  
Left Hand ◽  
Multiple Enchondromatosis ◽  
Cartilage Tumors ◽  
Ollier Disease ◽  
Surgery Department

Multiple enchondromatosis is a rare disease in which cartilage tumors appear at the level of the skeleton. The incidence is unknown due to the very few cases reported in world literature. We presented the case of a patient at the plastic surgery department at General hospital Dr. Ruben Leñero, otherwise healthy, referring first clinical manifestations at childhood with an increase in volume and deformity at the second and third fingers of the left hand.

scholarly journals Congenital Bronchobiliary Fistula: A Case Report and Literature Review

2021 ◽  
Vol 9 ◽  
Author(s):  
Zhen Bing ◽  
Rui Chen ◽  
Pengchao Xing ◽  
Yueyi Ren ◽  
Kefeng Hou
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Rare Disease ◽  
Thoracoscopic Surgery ◽  
Clinical Manifestations ◽  
Fiberoptic Bronchoscope ◽  
Bronchobiliary Fistula ◽  
Endobronchial Blocker

Congenital bronchobiliary fistula (CBBF) is a rare disease. Children with CBBF mostly have atypical clinical manifestations that can be easily missed. We report a case of a child with CBBF who was diagnosed with fistulography with the help of an endobronchial blocker and a fiberoptic bronchoscope. The CBBF was successfully removed by thoracoscopic surgery.

scholarly journals PRIMARY PANCREATIC LYMPHOMA WITH HERPES ZOSTER ASSOCIATED ACUTE PANCREATITIS: A CASE REPORT AND REVIEW OF LITERATURE.

2020 ◽  
pp. 49-51
Author(s):  
Vikram Chaturvedi ◽  
Rituparna Chaturvedi ◽  
Sharmistha Debnath ◽  
Abhishek Mishra
Keyword(s):  
Acute Pancreatitis ◽  
Abdominal Pain ◽  
Case Report ◽  
Herpes Zoster ◽  
Rare Disease ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
First Case ◽  
Pancreatic Lymphoma ◽  
Primary Pancreatic Lymphoma

Primary pancreatic lymphoma (PPL) is an extremely rare disease, with few cases reported in literature. Clinical manifestations are usually nonspecific and mimic other pancreatic diseases. Owing to paucity of data and experience of PPL, clinico-pathological features, differential diagnosis, optimal treatment and overall outcomes are not well known. Varicella zoster virus (VZV) is known to cause varicella in children and herpes zoster in adults. Acute pancreatitis caused by VZV is a rare and serious complication, with immunocompromised individuals being primarily affected. We report a case of VZV associated acute pancreatitis in a patient who was diagnosed and treated for primary pancreatic lymphoma, thus rendering her immunocompromised and susceptible to developing infectious pancreatitis. A 56 year old female patient presented with recurrent vomiting, upper abdominal pain and hyperaesthesia in the right upper quadrant of 2 days duration. Following admission her right sided abdominal pain worsened (stabbing pain) and a diagnosis of acute pancreatitis was confirmed based on elevated serum amylase levels and CT scan findings of pancreatic enlargement. The patient was managed conservatively with intravenous fluids, antibiotics, analgesics and topical acyclovir ointment. The patient continues to be asymptomatic after 5 years of follow up. Though extremely rare, a diagnosis of herpes zoster pancreatitis should be considered in such patients, and prompt treatment initiated to prevent further deterioration and to minimize mortality. Core tip: Primary pancreatic lymphoma is in itself a very rare disease, and our patient having presented with herpes zoster associated acute pancreatitis in a background of PPL makes this case even rarer. This is probably the first case report of herpes zoster associated acute pancreatitis in an immunocompromised patient due to primary pancreatic lymphoma.

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