What is the Lifespan for a Child with Holoprosencephaly?
Discussion
Holoprosencephaly (HPE) is a clefting problem of the brain. “[HPE] the result of incomplete or absent midline division of the embryonic forebrain into distinct cerebral hemispheres (prosencephalon) between the 18th and 28th day after conception.”
There are four distinct subtypes:
Alobar – both hemispheres are completely fused and are not separated into the left and right hemispheres. There is agenesis of the corpus callosum, arrhinencephaly and a single ventricle with fused thalami. Facial features are almost always affected.
Semilobar – the cerebral hemispheres are fused anteriorly bu...
Source: PediatricEducation.org - November 18, 2019 Category: Pediatrics Authors: Pediatric Education Tags: Uncategorized Source Type: news
What Are the Types of Tracheoesophageal Fistulas?
Discussion
Respiratory problems in infants can caused by many things including any type of obstruction from the nose to lung. Neonates and infants are particularly susceptible to changes in the size of the airway because of the physics of airflow resistance. Important reminders about infant airways:
Resistance = 1/radius4 (Poiseuille’s equation) therefore even a 1 mm decrease in the airway circumferences increases the airflow resistance x16.
Increased airflow causes less pressure along the walls, which can lead to collapse of the walls (Bernoulli’s and Venturi effects).
Neonates have smaller lungs relative to ...
Source: PediatricEducation.org - March 4, 2019 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
Healthy nurse spends all day with a nasogastric tube to empathise with her patients
Dorcas Boamah, 27, who works at Royal Free Hospital, wanted to be able to answer her patients honestly when they nervously asked her what it is like to have the tube fitted. (Source: the Mail online | Health)
Source: the Mail online | Health - January 30, 2019 Category: Consumer Health News Source Type: news
What Complications Can Gastrostomy Tubes Have?
Discussion
Gastrostomy tubes (GT or GTubes) have been used to support patients for about a century. They are placed between the abdominal skin and the stomach either percutaneously or surgically. The tubes can be a standard long tube with either a bumper or inflatable balloon internally and externally they have a retention piece to hold the GT in place. A button or low profile tube are similar but extend just beyond the skin.
Reasons for GT placement include:
Nutritional support
Hydration maintenance
Medication management
Aspiration avoidance
Gastric stasis decompression
Obstruction bypass
Quality of life improvement for...
Source: PediatricEducation.org - January 21, 2019 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
What is Genomic Imprinting?
Discussion
Silver-Russell syndrome (SRS) is a rare genetic syndrome first characterized by Silver in 1953 and Russell in 1954. Patients with SRS have characteristic growth patterns and clinical findings, although within an individual patient there are phenotypical differences. Patients are born small-for-gestational age (SGA) but have a relative macrocephaly. There is postnatal growth failure and difficulty feeding, with a very low body mass index. Body asymmetry (e.g. hemihypertrophy) and facial features (i.e. protruding forehead, triangular facies, micrognathia, dental anomalies, downturned mouth corners, and ear anomali...
Source: PediatricEducation.org - November 26, 2018 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
Recommendations Developed to Verify NGT Placement in Children
WEDNESDAY, Oct. 17, 2018 -- Recommendations on best practices for nasogastric tube (NGT) placement and verification in pediatric patients have been developed. The best practice recommendations were published online Sept. 6 in Nutrition in Clinical... (Source: Drugs.com - Pharma News)
Source: Drugs.com - Pharma News - October 17, 2018 Category: Pharmaceuticals Source Type: news
What is the Difference Between an Association and a Syndrome?
Discussion
VATER association was first described in the 1970s with additional congenital malformations being added to the association so it is most often called VACTERL association. It is a highly heterogeneous, overlapping condition estimated to occur in ~1/10,000-40,000 births. The cause is unknown. In animal models, some signaling pathway gene mutations have phenotypes of VACTERL association.
Experts suggest that patients having at least 2 components should be further evaluated (at least 3 for diagnosis) for VACTERL and other diseases in its differential diagnosis. There are more than 30 syndromes, mutations and diseas...
Source: PediatricEducation.org - February 5, 2018 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
The joy of cooking (and eating) after tracheomalacia
For most little kids, a trip to the supermarket is an annoying chore, made tolerable only by the opportunity to request sugary snacks as a reward. But when Charlotte McQueen accompanies her mother, Erin, to the store, it’s a journey marked by imagination and delight. “Mom, can we get that?” she asks, pointing to a can of pureed pumpkin. “Oh, and we’ll need marshmallows and we can make chocolate frosting. It will be a great cake!”
At nearly 5 years old, Charlotte is an avid baker — a talent she picked up not at culinary school, but at the Yawkey Family Inn. There, a volunteer taught her and other chronically i...
Source: Thrive, Children's Hospital Boston - January 10, 2018 Category: Pediatrics Authors: Jessica Cerretani Tags: Diseases & Conditions Our Patients’ Stories Dori Gallagher Dr. Michael Manfredi Dr. Russell Jennings Esophageal and Airway Treatment Center esophageal atresia and tracheoesophageal fistula posterior tracheopexy tracheomalacia Yawkey Fa Source Type: news
What is DIOS?
Discussion
Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane receptor (CFTR). It is found in the epithelium of the bronchi, intestine, pancreatic duct and biliary tree. It regulates chloride, bicarbonate and water secretion. The heterozygous state helps prevent against secretory diarrhea, but the homozygous state causes thickened secretions in the hollow tubes of the lungs and digestive tract. There are multiple mutations (> 2000) which have been currently classified into classes depending on their protein production and activity. CF patients generally are l...
Source: PediatricEducation.org - December 18, 2017 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
Prepared for the unexpected: How Henri beat C. diff
Henri and his sister, Lucienne
From the time he was born, Henri has been very reactive — to everything.
As a baby, he was allergic to milk and soy, which led to weeping eczema all over his body. His allergies meant frequent ear infections and sinus infections.
As a toddler, he was anemic and underweight. He had two urinary tract infections (UTIs) with fevers.
At age 3, he had a circumcision because of the repeated UTIs.
At age 4, a sinus infection spread to his eye orbit. Every sunscreen on the market gave him (and still gives him) a rash.
At age 5, a bug bite on his ear led to a cartilage infection that required antibio...
Source: Thrive, Children's Hospital Boston - November 29, 2017 Category: Pediatrics Authors: Marie Vedder Tags: Diseases & Conditions Our Patients’ Stories C-diff fecal transplant gastroenterology Lori Zimmerman Sonia Ballal Source Type: news
What Causes Microcephaly?
Discussion
Microcephaly is usually defined as an occipitofrontal head circumference (OFC) more than 2 standard deviations (SD) below the mean for sex, age and ethnicity. Severe microcephaly is used for OFC < 3 standard deviations. Rates of microcephaly range from 0.5-12 patients/10,000 live births.
The OFC should be measured at every well child visit and at other opportunities and plotted on standard growth charts. The OFC is measured using a nonelastic tape measure around the largest part of the head with the tape measure held above the eyebrows and ears. It is a highly reproducible measurement. There are several diff...
Source: PediatricEducation.org - September 25, 2017 Category: Pediatrics Authors: pediatriceducationmin Tags: Uncategorized Source Type: news
Advocating for Bridget: Coping with Hirschsprung ’s disease
On June 20, Bridget Landry celebrated a very special day with a hearty steak dinner. But it wasn’t her birthday. Instead, the 10-year-old and her family were marking the sixth anniversary of the day her ostomy bag was removed. As she enjoyed her meal, her parents, Carl and Laura, marveled at just how far their daughter had come. “For her first birthday, she couldn’t even take a bite of cake,” remembers Carl.
Bridget’s first few months of life were similar to those of most infants: She had a typical birth, nursed and met all her milestones. Yet at six months, her parents began to notice something was amiss. She wo...
Source: Thrive, Children's Hospital Boston - August 31, 2017 Category: Pediatrics Authors: Jessica Cerretani Tags: Diseases & Conditions Our Patients’ Stories Center for Advanced Intestinal Rehabilitation Dr. Samuel Nurko Dr. Tom Jaksic Hirschsprung's disease Motility and Functional Gastrointestinal Disorders Center Source Type: news
FDA Drug Safety Communication: Serious medication errors from intravenous administration of nimodipine oral capsules
[08-02-2010] The U.S. Food and Drug Administration (FDA) is alerting healthcare professionals that nimodipine capsules should be given ONLY by mouth or through a feeding tube (nasogastric tube). (Source: FDA Center for Drug Evaluation and Research - What's New)
Source: FDA Center for Drug Evaluation and Research - What's New - August 3, 2017 Category: Drugs & Pharmacology Source Type: news
Making progress: Eli is thriving after laryngeal cleft repair
Eli Bustard is pretty laid back for a 3-year-old — until he sees a picture of a dinosaur. “He’s obsessed,” laughs his mother, Nicole, who reports that Eli has been poring over a library book about these prehistoric beasts. Some of his other favorite pastimes: playing with trucks, caring for his Boston terrier and climbing up and down the musical stairs at Boston Children’s Hospital, which play a cheerful melody with every step.
A native of Bangor, Maine, Eli first came to Boston Children’s when he was just a few months old. Although he was born about three weeks early, he appeared to be in perfect health, and r...
Source: Thrive, Children's Hospital Boston - May 30, 2017 Category: Pediatrics Authors: Jessica Cerretani Tags: Diseases & Conditions Our Patients’ Stories Center for Airway Disorders Dr. Reza Rahbar laryngeal cleft Source Type: news
Draft nurse education plans include over 70 technical skills
More than 70 technical skills that newly qualified nurses should be competent in, including nasogastric tube insertion, injection of intravenous drugs, and urinary catheterisation, have been laid out in draft education plans being developed by the Nursing and Midwifery Council. (Source: Nursing Times)
Source: Nursing Times - March 15, 2017 Category: Nursing Source Type: news
