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Neonatal Teeth

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161. Hypophosphatasia: Enzyme Replacement Therapy Brings New Opportunities and New Challenges. (Full text)

expressed in the skeleton and developing teeth. In HPP,TNSALP substrates accumulate extracellularly, including inorganic pyrophosphate (PPi), an inhibitor of mineralization. The PPi excess can cause tooth loss, rickets or osteomalacia, calcific arthropathies, and perhaps muscle weakness. Severely affected infants may seize from insufficient hydrolysis of pyridoxal 5'-phosphate (PLP), the major extracellular vitamin B6 . Now, significant successes are documented for newborns, infants, and children

2017 Journal of Bone and Mineral Research PubMed abstract

166. Reducing the pain of childhood vaccination: an evidence-based clinical practice guideline

minutes. Coughing and gagging may occur but are relatively uncommon (= 5% of patients). 12 Sweet-tasting solutions are indicated for the management of painful procedures only, not for general comfort or as a food supplement. Given that the volume and the frequency of use are small, and given that many infants do not yet have teeth, the risk of dental caries is negligible. Although parents may wish to wash the infant’s mouth after the procedure, this may be irritating to the infant (...) held). However, parents instinctively pick up children when attempting to comfort them. 37 The systematic review 10 included four RCTs that examined the influence of the child’s position on the pain response dur- ing vaccination. 37–40 Altogether, 281 infants (from newborn to six months of age) and children (aged four to six years) were included. In three of the studies, 38–40 lying supine resulted in more pain than sitting upright or being held by a parent. One of these three studies involved

2011 CPG Infobase

167. Langerhans Cell Histiocytosis Treatment (PDQ®): Health Professional Version

million) and higher incidence in Hispanic patients (1.63 per 1 million) younger than 5 years. Crowded living conditions and lower socioeconomic circumstances were associated with a higher risk of LCH, possibly because of the correlation with maternal and neonatal infections.[ ] Identical twins and non-twin siblings with LCH, as well as LCH in multiple generations in one family, have been reported.[ ] Etiology The etiology of LCH is unknown. Risk Factors Although the following risk factors have been (...) also present with a generalized skin rash, which may mimic many other skin disorders and may or may not be pruritic. Vesicular LCH skin lesions need to be differentiated from congenital infections. Skin LCH in infants may be limited to skin (skin-only disease) or may be part of multisystem LCH. In a report of 61 neonatal cases from 1,069 patients in the Histiocyte Society database, nearly 60% (36 of 61 patients) had multisystem disease, and 72% of the patients with multisystem disease had risk

2016 PDQ - NCI's Comprehensive Cancer Database

168. Family Practice Notebook Updates 2017

in early pregnancy Newborn (neonatal abstinence syndrome) if maternal use No evidence of safety (gi, esophagus) Immune mediated esophagitis ( of the esophagus) that does not respond to management May present with solid , food impaction, anterior , and refractory Strongly associated with allergic conditions If management ineffective, try activating steroid MDI (e.g. HFA) and swallowing, not inhaling IV. Updates: October 2017 (geri, prevent) Approach falls as a sentinel event, a predictor of future falls (...) mortality (er, environ, heat) Exertional is on a spectrum from to to Other es include , and Removal from heat and rapid external cooling are the mainstays of initial care Not all patients are volume depleted (avoid over-hydration) (dental, teeth sx) In a normal appearing tooth (without exposed root or abscess), implies exposed dentin or pulp (cavity) Antibiotics are not indicated in where infection is absent (id, ) In the returning traveler, keep in the differential diagnosis (esp. immunocompromised

2018 FP Notebook

169. Congenital Syphilis

) , nariz en silla de montar sifilítica Derived from the NIH UMLS ( ) Ontology: Snuffles in newborn (C0456018) Concepts Disease or Syndrome ( T047 ) ICD10 SnomedCT 206325007 , 271375003 English Neonatal sniffles , Neonatal snuffles , Snuffles in newborn , Snuffles in newborn (finding) , newborn; snuffles , snuffles; newborn Dutch pasgeborene; verstopte neus , verstopte neus; pasgeborene Spanish coriza neonatal , resuello neonatal (hallazgo) , resuello neonatal Derived from the NIH UMLS ( ) Related (...) : Congenital Syphilis , Parrot Pseudoparalysis , Neonatal Snuffles , Congenital Syphilitic Pemphigus , Olympian Brow , Syphilitic Saddle Nose , Mulberry Molars , Higoumenakis Sign , Clutton's Joints , Saber Shin II. Epidemiology Congenital Syphilis in U.S. is increasing 2008: 8.1 cases per 100,000 live births III. Pathophysiology Transmitted transplacentally or via contact with syphilitic lesions during delivery IV. Risk Factors Children born to mothers who have sex with multiple partners Unique pocket

2018 FP Notebook

170. Family Practice Notebook Updates 2017

in early pregnancy Newborn (neonatal abstinence syndrome) if maternal use No evidence of safety (gi, esophagus) Immune mediated esophagitis ( of the esophagus) that does not respond to management May present with solid , food impaction, anterior , and refractory Strongly associated with allergic conditions If management ineffective, try activating steroid MDI (e.g. HFA) and swallowing, not inhaling IV. Updates: October 2017 (geri, prevent) Approach falls as a sentinel event, a predictor of future falls (...) mortality (er, environ, heat) Exertional is on a spectrum from to to Other es include , and Removal from heat and rapid external cooling are the mainstays of initial care Not all patients are volume depleted (avoid over-hydration) (dental, teeth sx) In a normal appearing tooth (without exposed root or abscess), implies exposed dentin or pulp (cavity) Antibiotics are not indicated in where infection is absent (id, ) In the returning traveler, keep in the differential diagnosis (esp. immunocompromised

2018 FP Notebook

171. Positive Pressure Ventilation

) Mask seal (e.g. beard) Obstruction Older Age No teeth (replace dentures for Bag Valve Mask Ventilation) Stiff lungs requiring increased y pressures ( , , , term pregnancy) References Difficult Airway Course IX. Technique Term Newborns Administer 5-8 ml/kg (15 to 25 ml per ventilation) Bag volume: 200 to 750 ml (usually >450 ml) Adults and older children Administer 10-15 ml/kg Hold mask over face with one hand Mask should fit snugly Covers mouth, nose and chin Should not cover eyes Thumb over nose (...) valve with finger during ventilation or Twist the pop-off valve into closed position Images Neonatal Ambu-Bag VII. Device: Noninvasive Positive Pressure Ventilation (NIPPV) (BiPAP) ( ) ( ) VIII. Precautions Do not use Bag Valve Mask to deliver free flow oxygen Oxygen only flows when squeezing bag Position patient for best ventilation ( , Levitan) Oxygen On Apply 15 lpm by under mask for Pull forward ( maneuver) Sit patient up (to 20 degrees) Anticipate difficult mask ventilation (Mnemonic: MOANS

2018 FP Notebook

172. Umbilical Vein Catheter

Catheter Aka: Umbilical Vein Catheter , Catheterization of Umbilical Vein II. Background Umbilical Vein Catheterization functions as a in newborns Umbilical vein remains patent for the first week of life (easiest to place the nearer to delivery) Catheter enters umbilical vein and ultimately enters ductus venosus (near liver) and inferior vena cava III. Indications: Emergent access to newborn circulation Acute stabilization Very ill infants (e.g. ) Very low birth weight (<750g) IV. Contraindications (...) Peritonitis V. Preparation Sterile set-up including drape, gloves, gown, mask Scalpel (#10 or #15 blade) and Scissors forceps without teeth, small clamps and needle holder Intravenous tubing with three way stopcock and Umbilical Vein Catheter 5 F for term infants (3.5 F for s) May place a warm compress over a dried umbilical stump to rehydrate it VI. Technique: Insertion Performed under sterile conditions Betadine or hibiclens preparation of the Draping of Prepare the Base of cord tied loosely

2018 FP Notebook

173. Infant Feeding

, Infant Nutrition , Neonatal Feeding , Neonatal Nutrition II. Monitoring Adequate newborn weight gain Anticipate up to 10% weight loss after delivery and regain to birth weight by 2 weeks Weight gain (1 gram = 0.0352 oz) Daily: 20-30 grams per day Weekly: 150-200 grams (5 to 7 ounces) per week Infant doubles birth weight in 6 months Adequate hydration Expect clear 6-8 times daily III. Protocol: Term Newborn Indications to delay start of enteral feeding Prolonged asphyxia with 5 minute less than 6 (...) weight for or Birth weight and still below reference growth chart weight at NICU discharge Neonatal dietician or Neonatologist Goals Infant feeds on demand every 2-4 hours Goal intake 120-150 ml/kg/day Ongoing growth approaches 50th percentile formula Inpatient (NICU): 24 calories/ounce used until weight >1800 grams Outpatient: Use 22 calories/ounce until one year of adjusted age or growth caught-up >25th percentile Use 20 calories/ounce after catch-up growth achieved up until one year

2018 FP Notebook

175. Hyperparathyroidism

) Familial Hyperparathyroidism (10-20%): Younger patients ( ) ( ) Hyperparathyroidism-Jaw Tumor Syndrome Neonatal severe Primary Hyperparathyroidism Other related causes Therapy External neck radiation exposure Neck surgery with injury or resection Tertiary Hyperparathyroidism Accelerated response to chronic (in advanced ) over-produces PTH causing V. Causes: Secondary Hyperparathyroidism Decreased calcium intake stage 4 or stage 5 and Decreased 1,25 dihydroxyvitamin D Normocalcemic Hyperparathyroidism (...) before Repeat serum testing in 2 weeks if normal Miscellaneous 1,25 Dihydroxyvitamin D3 Indicated if low and Phosphate ( ) Hyperphosphaturia tests IX. Imaging Classic XRay Findings "Salt and pepper" skull Distal Clavicle resorption Hand XRay Second and third middle phalange bone resorption Dental XRay Bone resorption of Lamina dura around teeth Sestamibi Technetium Tc 99mParathyroid Scan for localizing adenoma: 95% Causes of non-localizing scan Ectopic PTH production Diagnostic error Four-gland

2018 FP Notebook

176. Methamphetamine

Learning Motor slowing Psychiatric Changes Worsening s Depression with increased (esp. paranoia, s, s) Premature aging effect Chronic and associated , weight loss es (related to skin-picking behaviors) Severe ("meth mouth") Untreated in more than half of Methamphetamine users X. Adverse Effects: Pregnancy Fetus Prematurity and Heart defects Newborn withdrawal Abnormal sleep or food intake s Infant Methamphetamine is secreted in XI. Labs See See Comprehensive metabolic panel Serum phosphokinase Drug (...) mouth" - broken teeth and dry mouth Thinking and emotional problems NIH: National Institute on Drug Abuse Definition (NCI) An amphetamine with central nervous system (CNS) stimulating activity. Methamphetamine acts by both facilitating the release of catecholamines, particularly noradrenaline, dopamine and serotonin, from nerve terminals in the brain and by inhibiting their uptake. This leads to an increase in synaptic concentration of these neurotransmitters and results in increased stimulation

2018 FP Notebook

177. Langerhans Cell Histiocytosis Treatment (PDQ®): Health Professional Version

million) and higher incidence in Hispanic patients (1.63 per 1 million) younger than 5 years. Crowded living conditions and lower socioeconomic circumstances were associated with a higher risk of LCH, possibly because of the correlation with maternal and neonatal infections.[ ] Identical twins and non-twin siblings with LCH, as well as LCH in multiple generations in one family, have been reported.[ ] Etiology The etiology of LCH is unknown. Risk Factors Although the following risk factors have been (...) also present with a generalized skin rash, which may mimic many other skin disorders and may or may not be pruritic. Vesicular LCH skin lesions need to be differentiated from congenital infections. Skin LCH in infants may be limited to skin (skin-only disease) or may be part of multisystem LCH. In a report of 61 neonatal cases from 1,069 patients in the Histiocyte Society database, nearly 60% (36 of 61 patients) had multisystem disease, and 72% of the patients with multisystem disease had risk

2016 PDQ - NCI's Comprehensive Cancer Database

180. Methylthioninium chloride Proveblue

mg/kg which has sometimes induced haemolysis in this subpopulation (Guay 2009). The choice of the initial dose is confirmed by a study conducted on premature neonates A repeat dose (0.3 to 0.5 mg/kg body weight, i.e. 0.06-0.1 ml/kg body weight) may be given one hour after the first dose in cases of persistent or recurrent of symptoms or if methaemoglobin levels remain higher than normal. Extreme caution should be exercised when administering to newborns and infants below the age of 3 months due (...) literature shows that methylthioninium chloride can successfully be used in children with medicinal product induced-methaemoglobinaemia (table 5). The immature enzyme function in the newborn makes them more disposed for developing adverse events by the substance methylthioninium chloride itself. NADPH-methaemoglobin reductase in the newborn is approximately 60% of that in adults and reaches the normal adult concentration around 3 months of age. Also, infants younger than 3 months of age have higher

2011 European Medicines Agency - EPARs

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