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https://www.readbyqxmd.com/read/28928922/liver-transplantation-as-definitive-treatment-of-an-unresectable-mesenchymal-hamartoma-in-a-child-with-beckwith-wiedemann-syndrome
#1
Evelyn T Pan, Dor Yoeli, Michael L Kueht, N Thao N Galvan, Ronald T Cotton, Christine A O'Mahony, Abbas Rana, John A Goss
Mesenchymal liver hamartomas are benign tumors that can cause life-threatening abdominal distension and carry a risk for malignant transformation. In this case report, we describe a 13-month-old male with Beckwith-Wiedemann Syndrome (BWS) who presented with multiple mesenchymal liver hamartomas causing severe intra-abdominal mass effect. Imaging revealed six large multi-locular cystic lesions, ranging from 3.8 to 8.9 cm in diameter. The large size and spread of the tumors necessitated liver transplantation for complete removal...
August 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28765180/isolated-third-nerve-palsy-a-rare-neurological-presentation-of-burkitt-s-lymphoma
#2
Siddhesh Arun Kalantri, Akshatha Nayak, Saikat Datta, Maitreyee Bhattacharyya
Lymphoma patient presenting with isolated third nerve palsy is relatively rare, and diagnosis of underlying disease may be challenging. Until this date, less than 20 cases have been described in the literature. This is the case of a 3-year-old boy who presented to neurologist with ptosis of left eye for 8 days. On examination, abdominal mass was detected, and the child was referred to paediatric surgery department. Laparotomy and excision of the mass was done. Histopathology and immunohistochemistry of the mass confirmed it to be a case of Burkitt's lymphoma...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28752069/hemangioendothelioma-of-the-bladder-the-youngest-case-report-in-a-child
#3
Jupiter Sibarani, Dedi Rachmadi, Tjahjodjati, Nur Atik, Safendra Siregar, Laurina Armella, Sawkar Vijay
Hemangioendothelioma is a vascular tumor that commonly found in soft tissue, lungs, heart, liver, and bones, but it is very rare in bladder. We report a case of pediatric hemangioendothelioma of the bladder in a 2 years 7 months-old boy that treated with total excision of the bladder and bilateral ureterocutaneoustomy. This case is the second case in pediatric patients, and the youngest case that reported in a child. Our patient is doing well post operatively. But unfortunately interferon a-2b is not available in our hospital...
September 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28727974/autoamputation-of-the-appendix-presenting-as-a-calcified-abdominal-mass-following-necrotizing-enterocolitis
#4
Brent R Weil, Alyaa Al-Ibraheemi, Sara O Vargas, Shawn J Rangel
Autoamputation of the appendix has previously been reported in the literature, but it is likely an unusual event. We report a 2-year-old male child who had previously undergone laparotomy and bowel resection for necrotizing enterocolitis. Two years later a calcified intra-abdominal mass was identified on abdominal radiography and ultrasonography. Eventual laparotomy revealed a densely calcified mass within the transverse mesocolon. The mass was uneventfully resected. Pathologic evaluation showed appendiceal tissue, consistent with prior autoamputation of the vermiform appendix...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28707775/giant-fetal-hydrometrocolpos-associated-with-cloacal-anomaly-causing-postnatal-respiratory-distress
#5
Tatsuhito Kanda, Takashi Iizuka, Rena Yamazaki, Junpei Iwadare, Masanori Ono, Hiroshi Fujiwara
Persistent cloaca is a rare presentation wherein the urethra, vagina, and rectum converge into a common channel with a single perineal opening. Fetal hydrometrocolpos can result if fluid accumulates behind an obstruction of this common channel. A 29-year-old woman (G4P1021) was referred at 36 2/7 weeks of gestation for evaluation of a fetal abdominal cystic mass. Detailed ultrasonography and magnetic resonance imaging showed two symmetric cystic masses, bilateral hydronephrosis, and oligohydramnios. Elective cesarean delivery was performed at 37 0/7 weeks; the baby weighed 4043 g with Apgar scores of 5 and 6 at 1 and 5 min...
July 14, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28699604/torsion-of-a-wandering-spleen-in-an-adolescent-with-gaucher-disease
#6
Şükrü Güngör, Mehmet Öztürk, Fatma İlknur Varol, Ahmet Sığırcı, Mukadder Ayşe Selimoğlu
A wandering spleen is a rare condition characterized by the malposition of the spleen due to laxity or absence of its supporting ligaments. Although Gaucher disease generally presents with massive splenomegaly, which one of the predisposing causes of a wandering spleen, literature shows only one report of a wandering spleen in a child with Gaucher disease. In this case presentation, a 13-year-oldadolescent with Gaucher disease on enzyme replacement treatment was presented, who was detected having an abdominal mass on a routine visit and diagnosed with partial torsion of a wandering spleen associated with left lobe hypoplasia of the liver...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28694579/surgical-repair-of-congenital-abdominal-aortic-aneurysm-in-a-1-year-old-child-with-literature-review
#7
Ashwani Bansal, Aparajita Mitra, Akshay Kumar Bisoi, Sandeep Agarwala
Reported here is a case of 1-year-old male child who presented with huge abdominal mass, which on radiological investigation was diagnosed as retroperitoneal pseudoaneurysm of the aorta. On exploration, it was found to be a true aneurysm of infrarenal abdominal aorta with inflow agenesis. Aneurysm was excised, and aorta was reconstructed with 10 mm Dacron graft. Postoperative computed tomography angiography showed patent graft with good distal runoff. Literature review revealed that only 26 cases of congenital abdominal aortic aneurysm had been reported so far...
July 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28658845/large-multicystic-dysplastic-kidney-mimicking-a-large-cystic-renal-neoplasm
#8
Priyank Yadav, Sanjoy Kumar Sureka, M S Ansari, Rahul Soni, Hira Lal
Multicystic Dysplastic Kidney (MCDK) is one of the most common renal conditions seen in paediatric population. The natural history typically involves involution and many of the patients with unilateral disease may actually never become symptomatic. The initial evaluation is usually done on Ultrasonography (USG) while cross-sectional imaging and nuclear scan are reserved for diagnostic dilemmas. Management is conservative and surgery is done for selected patients with symptomatic cysts or suspicion of neoplasm...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28658778/pure-primary-extragonadal-retroperitoneal-yolk-sac-tumour-in-a-young-child-a-case-report
#9
Vineeth G Nair, H S Kiran, P R Shanthala
Germ Cell Tumours (GCTs) in children are uncommon, constituting approximately only 3% of all malignancies in children younger than 15 years of age. Primary extragonadal GCTs constitute only 1-5% of all GCTs and a retroperitoneal site is seen only in 4% of all extragonadal GCTs. Extragonadal GCTs arise from local transformation of primordial germ cells which have been misplaced during the migration of these cells through the midline dorsal mesentery in the fourth-sixth week of embryogenesis. GCTs in children show remarkable variability in age, site, presentation and histology...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28651694/maternal-plasma-phosphatidylcholine-polyunsaturated-fatty-acids-during-pregnancy-and-offspring-growth-and-adiposity
#10
Jonathan Y Bernard, Mya-Thway Tint, Izzuddin M Aris, Ling-Wei Chen, Phaik Ling Quah, Kok Hian Tan, George Seow-Heong Yeo, Marielle V Fortier, Fabian Yap, Lynette Shek, Yap-Seng Chong, Peter D Gluckman, Keith M Godfrey, Philip C Calder, Mary F F Chong, Michael S Kramer, Jérémie Botton, Yung Seng Lee
BACKGROUND: Polyunsaturated fatty acids (PUFA) are essential for offspring development, but it is less clear whether pregnancy PUFA status affects growth and adiposity. METHODS: In 985 mother-offspring pairs from the ongoing Singaporean GUSTO cohort, we analyzed the associations between offspring growth and adiposity outcomes until age 5 years and five PUFAs of interest, measured in maternal plasma at 26-28 weeks' gestation: linoleic acid (LA), arachidonic acid, α-linolenic acid, eicosapentaenoic acid, and docosahexaenoic acid (DHA)...
June 2017: Prostaglandins, Leukotrienes, and Essential Fatty Acids
https://www.readbyqxmd.com/read/28643234/body-mass-disorders-in-healthy-short-children-and-in-children-with-growth-hormone-deficiency
#11
Paweł Tomaszewski, Katarzyna Milde, Anna Majcher, Beata Pyrżak, Gul Tiryaki-Sonmez, Brad J Schoenfeld
The aim of the study was to determine the degree of adiposity and the incidence of body mass disorders, including abdominal obesity, in healthy short children and children with growth hormone deficiency. The study included 134 short children (height < 10th percentile) aged 7-15. In this cohort there were 63 (31 boys and 32 girls) children without diagnosed hormonal disorders and 71 patients (35 boys and 36 girls) with growth hormone deficiency. Basic somatic features were assessed and the study participants were categorized according to the percentage of body fat (%FAT), body mass index (BMI), and waist-to-height ratio (WHtR)...
June 23, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28586619/role-of-stretching-exercises-in-the-management-of-constipation-in-spastic-cerebral-palsy
#12
Waqar Ahmed Awan, Tahir Masood
BACKGROUND: Constipation is considered as one of the most common non-motor manifestations in cerebral palsy (CP). Along with other reasons, spasticity also contributes in developing constipation in CP, by decreasing mobility of trunk and lower extremities and abdominal viscera. Stretching exercises of upper extremities, trunk and lower extremities are routine management of spasticity in CP children. The objective of the study was to determine the role of stretching exercises in improving constipation symptoms in children with spastic cerebral palsy and to explore the association between spasticity and constipation among cerebral palsy children...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28538532/identification-and-management-of-obese-patients-in-a-pediatric-orthopaedic-clinic
#13
Natalia Davila, Joy Vess, Emily E Johnson
BACKGROUND: Childhood obesity is a complex healthcare problem that affects all aspects of a child's health. The American Academy of Pediatrics and the Expert Committee recommends that all children be evaluated for current medical conditions including the risk for obesity by identifying elevated body mass index (BMI), physical activity habits, and diet. Childhood obesity is defined as a BMI of 95th percentile or greater on standardized age-based growth charts. Abdominal and visceral fat mass has a negative effect on bone formation during childhood and adolescence...
May 2017: Orthopaedic Nursing
https://www.readbyqxmd.com/read/28445255/peutz-jeghers-syndrome-with-intermittent-upper-intestinal-obstruction-a-case-report-and-review-of-the-literature
#14
REVIEW
Shou-Xing Duan, Guang-Huan Wang, Jun Zhong, Wen-Hui Ou, Ma-Xian Fu, Fu-Sheng Wang, Shu-Hua Ma, Jian-Hong Li
RATIONALE: Peutz-Jeghers syndrome (PJS) is an autosomal dominant genetic syndrome characterized by a unique type of gastrointestinal hamartomatous polyp associated with oral and anal mucocutaneous pigmentations. Peutz-Jeghers polyps occur most numerously in the small intestine but frequently in the colon and stomach, only a few cases have been reported in the duodenum. PATIENT CONCERN: A further family history survey discovered 10 out of 14 members of the family (in 4 generations) had mucocutaneous pigmentations, but many of them were living in rural areas where they had no access to specialized medical services, so none were checked with endoscopy for polyps of hamartoma...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28440359/ultrasonographic-findings-in-hyperimmunoglobulin-d-syndrome-a-case-report
#15
Laura Otilia Damian, Otilia Fufezan, Mihaela Farcău, Simona Tătar, Călin Lazăr, Dorin-Ioan Farcău
Hyperimmunoglobulin D syndrome due to mevalonate kinase deficiency is a rare autoinflammatory disease with digestive tract involvement. We report an 11-year female child who has presented since the age of 1 year, bouts of fever, rash, joint swelling, pulmonary consolidation, lymph node involvement and hepatosplenomegaly. Hyperimmunglobulin D and increased urinary mevalonic acid were detected. The ultrasonographic features of hepatosplenomegaly ranged from increment in size to pseudotumoral involvement, with hypoechogenic masses without apparent wall...
April 22, 2017: Medical Ultrasonography
https://www.readbyqxmd.com/read/28423091/an-unusual-abdominal-wall-mass-in-a-child
#16
Amalia Maria do Espirito Santo Souza, Tomas Marzagão Barbuto, Flávia Alessandra Freitas, Nathalia Fernandes Vianna, Carla Maria Costa Zanchetta, Silvana Forsait, Claudio Borba, Alessandra Milani Prandini de Azambuja, Lilian Maria Cristofani, Vicente Odone
Abdominal tumors are one of the most common types of pediatric cancer. Therefore, they should always be included in the differential diagnosis of abdominal masses. Here, we present the case of a child whose initial hypothesis of diagnosis contemplated this possibility. Later, it was demonstrated that the abdominal mass found was secondary to a common parasitosis. A 2-year old, moderately malnourished and pale white boy was referred with a history of a rapidly growing, well-limited, middle abdominal mass. The mass was 10 by 3 cm, hard and poorly movable, apparently involving both abdominal rectus muscles...
April 13, 2017: Revista do Instituto de Medicina Tropical de São Paulo
https://www.readbyqxmd.com/read/28364513/sleep-duration-and-adiposity-in-early-childhood-evidence-for-bidirectional-associations-from-the-born-in-bradford-study
#17
Paul J Collings, Helen L Ball, Gillian Santorelli, Jane West, Sally E Barber, Rosemary Rc McEachan, John Wright
Study Objectives: To examine independent associations of sleep duration with total and abdominal adiposity, and the bidirectionality of these associations, in a young biethnic sample of children from a disadvantaged location. Methods: Child sleep duration (h/day) was parent-reported by questionnaire and indices of total (body weight, body mass index, percent body fat (%BF), sum of skinfolds) and abdominal adiposity (waist circumference) were measured using standard anthropometric procedures at approximately 12, 18, 24, and 36 months of age in 1,338 children (58% South Asian; 42% White)...
February 1, 2017: Sleep
https://www.readbyqxmd.com/read/28337353/gastric-duplication-a-rare-cause-of-recurrent-vomiting
#18
Brahmananda Koduri, Katie McHale, Christina Yost, Michael H Goodman, Dennis Hoelzer
Vomiting is a physical finding that can occur at any age but presents the greatest challenge when it is recurrent in a child. The etiology is varied (Sieunarine and Manmohansingh, 1989; Suzuki, 1982), and recurrent vomiting can be a symptom of life threatening medical or surgical emergencies. Early recognition is mandatory for preventing delay in management and potential complications. Gastric duplication is rare and mostly diagnosed in infancy with only a few cases documented in the medical literature presenting in childhood...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28299818/rapid-weight-gain-during-infancy-and-early-childhood-is-related-to-higher-anthropometric-measurements-in-preadolescence
#19
H Nanri, T Shirasawa, H Ochiai, S Nomoto, H Hoshino, A Kokaze
BACKGROUND: This study examined the relationship between rapid weight gain during infancy and/or early childhood and anthropometric measurements [body mass index (BMI), percent body fat (%BF), waist circumference (WC) and waist-to-height ratio (WHtR)] in preadolescence by sex. METHODS: Subjects were fourth-grade school children (aged 9 to 10 years) from elementary schools in Ina-town, Japan, in 2010. Measurements of height, weight, %BF and WC were conducted for each subject...
March 16, 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/28284198/multicystic-nephroma-masquerading-as-hydatid-cyst-a-diagnostic-challenge
#20
Abdelmoneim E M Kheir, Aziza M Elnaeema, Sara M A Gafer, Sawsan A Mohammed, Mustafa E Bahar
BACKGROUND: Multicystic nephroma is an uncommon, non-familial renal neoplasm that is usually benign. About 200 cases of this lesion have been described in the literature. CASE PRESENTATION: We report on a Sudanese child who presented at the age of two and a half years with an abdominal mass, clinical and radiological features favored the diagnosis of hydatid cyst which is endemic in this African tropical country, and the diagnosis of multicystic nephroma was only possible after histopathological examination...
March 11, 2017: BMC Urology
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