scholarly journals Cronkhite-Canada Syndrome Successfully Treated by Corticosteroids before Presenting Typical Ectodermal Symptoms

2020 ◽  
Vol 14 (3) ◽  
pp. 561-569
Author(s):  
Kazumoto Murata ◽  
Kiichi Sato ◽  
Shinya Okada ◽  
Daisuke Suto ◽  
Takaaki Otake ◽  
...  
Keyword(s):  
Weight Loss ◽  
Family History ◽  
Corticosteroid Therapy ◽  
Terminal Ileum ◽  
Chronic Diarrhea ◽  
Endoscopic Examination ◽  
Hyperplastic Polyps ◽  
Gastrointestinal Polyposis ◽  
History Of ◽  
Gastric Polyposis

Cronkhite-Canada syndrome (CCS) is a rare disease characterized by diffuse gastrointestinal polyposis with chronic diarrhea and ectodermal change, but its etiology is unknown. We present a case at the age of 26 years complaining of epigastralgia and weight loss. Endoscopic examination revealed extensive diffuse polypoid lesions of the stomach and the terminal ileum, all of which showed hyperplastic polyps pathologically. There were no polypoid lesions in his colon. He has no family history of diffuse gastrointestinal polyposis. Diffuse gastrointestinal hyperplastic polyposis without any hereditary association led us to suspect this case as CCS although he did not show chronic diarrhea and any ectodermal symptoms such as onychodystrophy, alopecia, and hyperpigmentation. After initiation of a corticosteroid therapy, his epigastralgia disappeared and he gained appetite and weight, accompanied by normalization of serum albumin levels. Endoscopic examination 1 year after initiation of corticosteroid therapy revealed a decrease in the number of gastric polyposis and those inflammations. This rare young case may suggest that early therapeutic intervention with corticosteroids could improve the prognosis of CCS, preventing not only malnutrition but also appearance of several ectodermal symptoms.

Differences in Mechanisms Between Weight Reduction Sensitive and Insensitive Blood Pressure Reduction in Obese Subjects

Hypertension ◽  
2000 ◽  
Vol 36 (suppl_1) ◽  
pp. 725-725
Author(s):  
Kazuko Masuo ◽  
Hiroshi Mikami ◽  
Toshio Ogihara ◽  
Michael L Tuck
Keyword(s):  
Weight Loss ◽  
Family History ◽  
Weight Reduction ◽  
Blood Pressure Reduction ◽  
Study Groups ◽  
Plasma Norepinephrine ◽  
Ang Ii ◽  
Major Mechanism ◽  
History Of ◽  
History Of Obesity

P180 This study was conducted to clarify the differences in mechanisms between weight reduction (WR) sensitive and insensitive BP reduction, and to evaluate the contribution of family history of obesity (FH) to WR-induced BP reduction. In 61 obese hypertensive men (HT, 28.1±0.9 kg/m2, 35±3 years, 171±6/106±5 mmHg) and 52 obese normotensive men (NT, 27.9±0.6 kg/m2, 34±4 years, 131±5/83±4 mmHg), BMI, BP, fasting plasma norepinephrine (NE), angiotensin II (Ang II), PRA, leptin, insulin were measured every 2 week for 24 weeks with weight loss program (low caloric diet 1000kcal, 7gNaCl + excercise≥1 hr/day). WR and WR sensitive BP reduction were defined as >10% reduction in BMI or mean BP at week 12. 64% of HT and 63% of NT succeeded in WR, and 59% of HT with WR (sensitive vs insensitive P<.05) and 70% of NT with WR (P<.01) were sensitive in BP reduction. When FH+ was defined as at least one parent was obese (BMI>27.0 kg/m2), prevalence of FH+ was higher in 86% of HT and 95% of NT who failed in WR, and higher in 94% of HT and 80% of NT with WR insensitive BP reduction. Only the subjects who succeeded in WR were analyzed in this study. At entry, BP, NE, Ang II, PRA and insulin were higher in HT than in NT, although BMI and leptin were similar. However, the parameters at entry were similar between WR sensitive and insensitive BP reduction in each NT and HT. The decrements (Δ) in BP, NE, Ang II, leptin,insulin were significantly greater in subjects with WR sensitive BP reduction than subjects with WR insensitive BP reduction regardless of BP status during the study, although ΔBMI was similar. Significant decreases in the parameters were noted in earlier period in subjects with WR sensitive BP reduction than in subjects with insensitive BP reduction, and in NT than in HT. In the 4 study groups regardless of BP status or WR induced BP reduction, the decrease in NE preceded BP decline, and the decreases in Ang II, insulin, leptin & PRA followed BP decline with WR. These results suggest that a family history of obesity appears to contribute closely to resistance in weight loss and also to WR insensitive BP reduction. Suppression on sympathetic overactivity is a major mechanism in WR induced BP reduction.

scholarly journals Small Intestine Large Granular Lymphoma in a Horse

2001 ◽  
Vol 38 (2) ◽  
pp. 223-226 ◽  
Author(s):  
P. Herraez ◽  
B. Berridge ◽  
P. Marsh ◽  
B. Weeks ◽  
F. Ramiro-Ibañez
Keyword(s):  
Weight Loss ◽  
Small Intestine ◽  
Tumor Cells ◽  
Phosphotungstic Acid ◽  
Chronic Diarrhea ◽  
Medical Center ◽  
Cell Marker ◽  
Regional Lymph Nodes ◽  
History Of ◽  
Neoplastic Lymphocytes

A 12-year-old Appaloosa gelding was referred to the Texas Veterinary Medical Center with a history of chronic diarrhea and weight loss. At necropsy, numerous oval, craterlike ulcers were observed throughout the small intestine. Histologically, these lesions were composed of a neoplastic proliferation of round cells with intracytoplasmic phosphotungstic acid-hematoxylin-positive granules. The tumor cells stained positively for the CD3 antigen and negatively for a B-cell marker. A diagnosis of large granular lymphoma was based on the morphologic and immunohistochemical characteristics of the neoplasm. The postmortem presentation of this case depicted unusual multifocal, ulcerative lymphomatous lesions throughout the small intestine without involvement of the regional lymph nodes. The histologic and ultrastructural morphology of the neoplastic lymphocytes was similar to that in previously reported cases of abdominal equine large granular lymphomas, but in this case the neoplasm was restricted to the small intestine.

scholarly journals Evaluation of The 10 Warning Signs In Immunodeficient Patients: Additional Suggestions

2021 ◽  
Author(s):  
Fadime Ceyda Eldeniz ◽  
Yahya Gül ◽  
Alaattin Yorulmaz ◽  
Şükrü Nail Güner ◽  
Sevgi Keles ◽  
...  
Keyword(s):  
Family History ◽  
Early Diagnosis ◽  
Chronic Diarrhea ◽  
Warning Signs ◽  
Control Group ◽  
Healthy Children ◽  
Physician Awareness ◽  
History Of ◽  
And Control ◽  
Immunological Evaluation

Abstract Objective: Ten warning signs of primary immunodeficiency (PID) were suggested by the Jeffrey Modell Foundation (JMF), to increase physician awareness of PID. These warning signs have not yet been evaluated for patients with secondary immunodeficiency (SID). This study investigated whether the 10 warning signs used for the diagnosis of PID are sufficient for the diagnosis of SID, and explored the possibility of additional signs.Methods: This prospective study was conducted between June and December 2020. The mothers of 162 patients with PID and SID, and mothers of 200 healthy children, were asked to complete a questionnaire about family and personal history in addition to the warning signs of PID developed by the JMF. A JMF score was created by giving one point for each “Yes” answer for the 10 warning signs of PID. Medical records of the patients were evaluated for possible additional warning signs for PID and SID. Results: The JMF scores of the PID (3.36 ± 1.65) and SID (3.72 ± 1.12) groups were significantly higher than the scores of the control group (0.34 ± 0.61) (p < 0.05). A sign for immunological evaluation in two patients without warning signs in the PID group was found to be chronic diarrhea. In addition to the 10 JMF warning signs, we found that consanguinity and a family history of tuberculosis were statistically significant in our PID group, compared with the SID and control groups. Conclusions: The JMF warning signs are important for early diagnosis of PID. Our study showed that these signs may also be used for the early diagnosis of SID in patients and, according to our results, in addition to the 10 JMF signs for PID, parental consanguinity, chronic diarrhea, and a family history of tuberculosis may also be considered warning signs for the early diagnosis of PID.

scholarly journals A Rare Case of Chronic Diarrhea in a Pediatric Patient

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A988-A988
Author(s):  
Priya S Srivastava ◽  
Kevin Yen ◽  
Robert Lindquist ◽  
Arun Rangaswami ◽  
Doruk Ozgediz ◽  
...  
Keyword(s):  
Family History ◽  
Neuroendocrine Tumor ◽  
Vasoactive Intestinal Peptide ◽  
Neuroendocrine Tumors ◽  
Chronic Diarrhea ◽  
Uterine Cancer ◽  
Secretory Diarrhea ◽  
Watery Diarrhea ◽  
History Of ◽  
Well Differentiated

Abstract Background: VIPoma, also known as Watery Diarrhea-Hypokalemia- achlorhydria (WDHA) Syndrome is a rare manifestation of multiple endocrine neoplasia syndrome type 1 (MEN1). Vasoactive intestinal peptide, part of the secretin-glucagon family, may be overexcreted in tumors associated with MEN1 and results in diarrhea that persists while fasting, resulting in massive secretion of water and electrolytes. First-line treatment is surgical resection. Clinical Case: We present a 13-year-old male with a past medical history of chronic diarrhea for four years who was transferred from an outside hospital for severe diarrhea and associated electrolyte derangements, including hypokalemia of &lt;1.0 mmol/L (3.5-5.0 mmol/L), sodium of 120 (135-145 mmol/L), and chloride of 84 mmol/L (101-110 mmol/L). Family history was significant for pancreatic, breast, thyroid, stomach, parathyroid, and uterine cancer, as well as hyperparathyroidism and nephrolithiasis. The patient had been admitted to the hospital before for a similar episode of acute on chronic diarrhea but was treated for infectious diarrhea during that admission. CT enterography was obtained during this hospitalization and it revealed multiple solid and heterogeneous appearing pancreatic masses in the head and tail of the pancreas. Work-up was significant for pancreatic polypeptide of 1,523 pg/mL (92-752 pg/mL) and vasoactive intestinal peptide of 1,105 pg/mL (&lt;75 pg/mL). Pancreatic biopsy revealed a grade 2 pancreatic neuroendocrine tumor. Genetic testing revealed a known pathogenic mutation in the menin gene p.R526 (c. 1579&gt;T, pArg527). A pylorus-preserving total pancreatectomy, duodenectomy, cholecystectomy, and splenectomy was performed and surgical pathology revealed a well-differentiated grade 1 neuroendocrine tumor in the head of the pancreas and a well-differentiated grade 2 neuroendocrine tumor in the tail of the pancreas. Since the surgery, the patient currently has no signs of other neuroendocrine tumors associated with MEN1 but continues to follow-up for regular screening for other tumors associated with MEN1. Clinical Lessons: 1. VIPoma, also known as Watery Diarrhea- Hypokalemia- Achlorhydria Syndrome, is characterized by secretory diarrhea that persists while fasting. 2. VIPoma should be considered in patients with a history of chronic diarrhea and a significant family history of neuroendocrine tumors.

Megaloblastic Anemia Characterized by Microcytosis: Imerslund-Grasbeck Syndrome With Coexistent α-Thalassemia

PEDIATRICS ◽  
1988 ◽  
Vol 81 (6) ◽  
pp. 875-876
Author(s):  
CAROLYN L. RUSSO ◽  
PAUL E. HYMAN ◽  
RONALD S. OSEAS
Keyword(s):  
Case Report ◽  
Family History ◽  
Vitamin B12 ◽  
Autosomal Recessive ◽  
Chronic Diarrhea ◽  
Megaloblastic Anemia ◽  
Microcytic Anemia ◽  
History Of ◽  
Autosomal Recessive Condition ◽  
Vitamin B12 Malabsorption

Imerslund-Grasbeck syndrome is a hereditary autosomal recessive condition of selective vitamin B12 malabsorption in the terminal ileum, resulting in chronic megaloblastic anemia.1,2 The purpose of this report is to describe a child with coincident Imerslund-Grasbeck syndrome and α-thalassemia, who also had a vitamin B12-sensitive microcytic anemia. CASE REPORT When R.S. was 15 months of age his mother reported that progressive anorexia and lethargy had developed. There was no history of chronic diarrhea, vomiting, frequent infections, or pica; there was no family history for blood dyscrasias. R.S. was a black boy who weighed 8.3 kg (less than the fifth percentile); his length was 74 cm (less than the fifth percentile), and his head circumference was 45 cm (second percentile).

scholarly journals An 11-year-old boy with chronic diarrhea and weight loss

2020 ◽  
Vol 13 (1) ◽  
pp. 35-41
Author(s):  
Maimuna Sayeed ◽  
Nazmul Hassan ◽  
Md. Rukunuzzaman ◽  
Bishnu Pada Dey
Keyword(s):  
Weight Loss ◽  
Body Weight ◽  
Fecal Incontinence ◽  
Small Volume ◽  
Chronic Diarrhea ◽  
Significant Weight Loss ◽  
History Of

This article has no abstract. The first 100 words appear below: An 11-year-old boy presented with 15 months history of diarrhea with up to 10-20 times small volume stool daily with mucus. It was associated with a feeling of incomplete defecation and excessive straining. The frequency of diarrhea increased to 20-30 times per day over the last 8 months. Subsequently, he developed fecal incontinence, for which he couldn't attend his school. He also had a history of significant weight loss in the last 7 months. According to his mother's statement, his body weight was reduced to 64 kg from 77 kg during this illness.

scholarly journals The Demographic and Clinical Characteristics of Ulcerative Colitis in a Northeast Brazilian Population

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Bruno César da Silva ◽  
Andre Castro Lyra ◽  
Carlos Maurício Cardeal Mendes ◽  
Camila Paula Oliveira Ribeiro ◽  
Sonyara Rauedys Oliveira Lisboa ◽  
...  
Keyword(s):  
Weight Loss ◽  
Family History ◽  
Steroid Therapy ◽  
Cross Sectional Study ◽  
Immunosuppressive Drugs ◽  
Age At Diagnosis ◽  
Cross Sectional ◽  
Younger Age ◽  
History Of ◽  
Rural Patients

Introduction. The purpose of this study was to describe the clinical and demographic characteristics of UC in Bahia, a Brazilian state, and to identify the variables associated with extensive colitis, steroid therapy, immunosuppression, and colectomy.Methods. In this cross-sectional study UC patients were interviewed, and additional information was collected from the medical records. Descriptive statistics and multivariate Poisson regression analysis were used.Results. This study included 267 individuals, the mean age of whom was 39.4 years at diagnosis. There was a predominance of females and left-side colitis. Extensive colitis was positively associated with male gender, diarrhea, weight loss, and a younger age at diagnosis. In contrast, active smoking and a family history of IBD were negatively associated with extensive colitis. Positive associations were observed between steroid therapy and diarrhea, weight loss, urban patients, extraintestinal manifestations (EIMs), and hospitalization. Younger age and weight loss at diagnosis, a family history of IBD, extensive colitis, EIMs, hospitalization, and steroid therapy were all positively associated with immunosuppression. In contrast, Caucasian individuals, smokers, patients with rectal bleeding, and rural patients areas were all observed to have a decreased likelihood of immunosuppression.Conclusions. Our results corroborate the association between higher prevalence of extensive colitis and younger age at diagnosis. An association between steroid therapy and clinical presentation at diagnosis was observed. The observation that white individuals and rural patients use less immunosuppressive drugs highlights the need to study the influence of environmental and genetic factors on the behavior of UC in this population.

P1697MULTI- NATIONAL SURVEY AMONG NEPHROLOGISTS AND TRANSPLANT SURGEONS ABOUT THE SUITABILITY AND ACCEPTABILITY OF MARGINAL LIVE KIDNEY DONORS WITH OBESITY OR ABNORMAL BLOOD SUGAR PROFILE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Ziad Arabi ◽  
Mohammad Bukhari
Keyword(s):  
Bariatric Surgery ◽  
Weight Loss ◽  
Family History ◽  
Blood Sugar ◽  
Kidney Donation ◽  
Morbidly Obese ◽  
Kidney Donors ◽  
Living Kidney Donors ◽  
History Of ◽  
Sugar Profile

Abstract Background and Aims There are no clear guidelines about the suitability and acceptance of living- kidney donors with obesity or abnormal blood sugar profile. Method To form a consensus about the acceptance of these donors, a survey was distributed to nephrologists and transplant surgeons in different countries and through AST and ERA-EDTA. Results Of a total of (n=122) respondents from 22 countries: 80% (N= 96) were nephrologists and 20% (N=23) were transplant surgeons. The majority were heavily involved in pre-transplant evaluations of donors and recipients and have &gt; 6 years in practice. 75% of the respondents will accept obese donors (BMI 30-35) if donors show some weight loss before donation or at least show commitment to lose weight in the future. However, the presence of a strong family history of diabetes ( DM) in obese donors (even with normal fasting blood sugar (FBS) and hemoglobin A1C [ Hgb A1C]) mandates weight lose preferably to BMI of 30 ( n=46, 38% ) or at least to lose some weight before being considered for donation (n=30, 25%). On the other hand, morbidly obese donors (BMI 36-40) with normal FBS, Hgb A1C and negative family history of DM thought to need to decrease their weight to BMI 30 ( 47% of the respondents) or at least lose some weight before being considered for donation ( n=30, 25%). However, 22% of the respondents would decline these donors due to the risk of relapse of obesity. If an obese donor started to have impaired fasting blood sugar ( IFG), the majority (47%) will decline him/her because of the higher risk to develop DM. (33%) will delay the donation till BMI reaches below 30 and IFG is resolved as a result of weight reduction. Most of the respondents (52%) will not rely solely on isolated mildly elevated Hgb A1C (e.g. Hgb A1C = 5.7- 6) or isolated (IFG) as the critical indicator to decline a young donor with normal weight and normal FBS. Instead 2hrs- glucose tolerance test will be indicated. The presence of even mild and controlled diabetes in a middle age (e.g. 55 years old donor) thought to be a contraindication for kidney donation in 62% of the respondents. However, such donors might be allowed to donate if no alternative donor is available after clearly explaining the risks (23%). In regard to resolved DM after bariatric surgery and weight loss, 43% of the respondents said they will consider them for donation if two years passed after bariatric surgery without obesity relapse (43%). Up to one forth may consider these donors earlier (once BMI is below 30). Despite the surgical difficulties related to obesity in kidney donors, transplant surgeons seem more likely than nephrologists to accept obese donors (P= 0.046), morbidly obese ( P=NS). Conclusion Living kidney donors with isolated obesity, (IFG), mildly abnormal HgbA1C or history of bariatric surgery are still considered for kidney donation by many centers. However, donors with more than one abnormality especially at young age are not considered for donation.

Sign in / Sign up

Export Citation Format

Share Document