Congenital pyloric atresia – nine new cases: Single-center experience of the long-term follow-up and the lessons learnt over a decade

Kansra, Monal; Raman, Vijaya; Kishore, Kamal; Khanna, Sudhansoo; Puri, Bipin; Sharma, Abhishek

doi:10.1016/j.jpedsurg.2018.04.015

Cited by 13 publications

(4 citation statements)

References 14 publications

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“…7Also, in study of nine cases with the Congenital Pyloric Atresia (CPA), M.Kansra et al had one patient with the CPA (type 3) and the ileal atresia associated with the EB. (8) In our case the EB is in combination with congenital skin aplasia, which is based on the author Frieden, in 6th group and it is called Bart's syndrome. The inheritance pattern of Bart's syndrome appears to be an autosomal dominant.…”

Section: Discussionmentioning

confidence: 90%

Congenital Absence of Skin on the Right Leg and Nail Abnormalities-Epidermolysis Bullosa or Bart’s Syndrom ?

Stanojevic

Prokic

Savic

et al. 2021

Serbian Journal of Experimental and Clinical Research

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Children born with the epidermolysis bullosa (so-called “butterfly children”) can eat only liquid or soft food due to the blisters on their mouth, tongue and esophagus. Due to their inactivity and permanent wounds, their fingers are curved and grown with a fist. Their eyes, anus and genitals are not spared either. The digestion is usually poor, so they often suffer from the constipation, and sometimes the intestine discharge can be performed only surgically. Due to frequent and numerous wounds, infections may develop, which can lead to sepsis. Wounds are caused by any kind of the pressure and re-bandaging of wounds is the most painful. These children can later be susceptible to other diseases, especially the skin cancer. More than 80% of children diagnosed with this disease become disabled in the first years of their lives, and some of them pass away immediately after birth. The average lifespan of the diseased is about 28 years. Here we have presented a rare case of a newborn male infant with a dystrophic epidermolysis bullousa, a congenital skin aplasia on the right leg and a nail dystrophy. Based on a typical clinical presentation, we think that it is Bart’s syndrome.

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Section: Discussionmentioning

confidence: 90%

Congenital Absence of Skin on the Right Leg and Nail Abnormalities-Epidermolysis Bullosa or Bart’s Syndrom ?

Stanojevic

Prokic

Savic

et al. 2021

Serbian Journal of Experimental and Clinical Research

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“…The article by Webb LJ et al ., published in the QJM: An International Journal of Medicine journal in 1979, has the highest total citation count This article is an original clinical research work dealing with the presentation, management, and outcome of EHPVO. [ 19 ] When the total citation per year was taken into consideration, the article “Noncirrhotic portal hypertension-Diagnosis and management” was authored by Khanna and Sarin and published in 2014 in the Journal of Hepatology was the highest (19.1). [ 18 ] This article was a narrative review and covered various aspects of EHPVO in great detail.…”

Section: Discussionmentioning

confidence: 99%

Bibliometric analysis and visualization of the extrahepatic portal venous obstruction publication landscape

Dhua,

Garg,

Yadav

et al. 2023

J Indian Assoc Pediatr Surg

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A BSTRACT Introduction: A scientometric analysis was conducted to characterize the global research publications in extrahepatic portal venous obstruction (EHPVO), and state-of-the-art visualization graphics were generated to provide insight into specific bibliometric variables. Materials and Methods: The Web of Science database was accessed for research productivity and bibliometric variables of countries, institutions, authors, journals, and content analysis of top-20 cited documents were performed. Collaborative networks and co-occurrence of keywords map were generated using VOSviewer software. Results: Two hundred and sixteen records were retrieved with an annual growth rate of 2.53%. India is the leading country in productivity ( n = 4339), followed by the USA and China. Post Graduate Institute of Medical Education and Research, Chandigarh, was the top productive institute. Sarin SK was the most prolific author, having the highest citations received and h-index. The hotspot topics were “portal hypertension,” “cirrhosis,” “children,” “biliopathy/cholangiopathy,” “liver fibrosis,” and “liver transplantation” as per keyword co-occurrence networking. J Gastroenterol Hepatol had the most publications of EHPVO research as well the h-index. Regarding collaborative network mapping, the USA and Primignani M were the significant nodes among country and author, respectively. Conclusion: EHPVO research publication volume is low but is gradually progressing with dominant contributions from Indian institutes and authors. Most highly cited articles are of low level of evidence, and multi-institutional collaborative research can be the way forward.

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“…Delayed presentation is possible and could be due to a partial obstruction causing a subtle course of the disease, as well as later presentation of the symptoms, absence of primary health care facilities, and possible ignorance of this rare entity, as reported in Kansra et al's study that showed the median age at the onset of symptoms was 6 months (1 day to 36 months) and the median age at presentation to the hospital was 7 months (1 day to 44 months) with a 5-month mean delay between them. 12 The diagnosis of PA cases is straightforward and can be achieved through patient history, physical examination, and plain abdominal X-ray. An exception is a perforated web causing a partial intestinal obstruction.…”

Section: Discussionmentioning

confidence: 99%

Delayed Diagnosis of a Pyloric Web Causing Gastric Outlet Obstruction in a 13-Month-Old Girl

Elifranji

Sankar

Abdelrasool

et al. 2021

European J Pediatr Surg Rep

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Pyloric web is a rare cause of gastric outlet obstruction. Classical pyloric web can be diagnosed by obtaining a patient history, physical examination, and plain abdominal X-ray, whereas a perforated web leads to incomplete intestinal obstruction. Delayed diagnosis is rare, and the definite diagnosis is made by upper endoscopy. In this report, we report a case of a girl in whom a pyloric web was diagnosed at the age of 13 months.

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Congenital pyloric atresia – nine new cases: Single-center experience of the long-term follow-up and the lessons learnt over a decade

Cited by 13 publications

References 14 publications

Congenital Absence of Skin on the Right Leg and Nail Abnormalities-Epidermolysis Bullosa or Bart’s Syndrom ?

Congenital Absence of Skin on the Right Leg and Nail Abnormalities-Epidermolysis Bullosa or Bart’s Syndrom ?

Bibliometric analysis and visualization of the extrahepatic portal venous obstruction publication landscape

Delayed Diagnosis of a Pyloric Web Causing Gastric Outlet Obstruction in a 13-Month-Old Girl

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