Which metabolic disease presents with retinal hemorrhage and intracranial bleeding and can be mistaken for child abuse? ✅ Glutaric aciduria type 1 #PedsICU Why?🤔 ⊖ enzymes involved in FA metabolism, leading to ⬇️ synthesis of myelin and weakened blood vessels in the brain 🧠

▪ While Beta blocker toxicity tends to cause hypoglycemia and altered mental status, calcium channel blocker toxicity is usually associated with hyperglycemia and mental status is preserved.

Since the introduction of Dantrolene, the mortality rate in Malignant Hyperthermia went down from 70% in 1960s to about 15%. Dantrolene should be administered without delays. New Episode 🔗: shorturl.at/gpAKX Management of MH in the PICU! #PedsICU

▪Malignant Hyperthermia susceptibility (MHS) is autosomal dominant (AD) condition with variable penetrance and expressivity. ▪High index of suspicion especially in the setting of positive family history is critical.

It's an honor to be featured with an international cohort of audio-learning educators on the most recent OPENPediatrics World Shared Practice Forum Podcast! Here's a compiled list of the most popular PICU podcasts: bit.ly/4bbDrjM #PedsICU

The four main causes of hypoxemia are: ▪ Alveolar hypoventilation: Normal A-a gradient ▪ Diffusion defect: High A-a gradient ▪ V/Q mismatch: High A-a gradient ▪ Anatomic right to left shunt: High A-a gradient - supplemental oxygen is ineffective in correcting

❓Which metabolic condition associated with respiratory alkalosis and hyperammonemia? ✅Urea Cycle Defects. Of the most common types; ▪OTC (ornithine transcarbamylase) deficiency is X-linked recessive with high orotic acid levels. ▪CPS (Carbamoyl phosphate synthetase)

▪ Kussmaul breathing in urea cycle defects might result in respiratory alkalosis. ▪ Ammonul is a combination of sodium phenylacetate and sodium benzoate. ▪Ammonul is used as nitrogen scavenger to treat hyperammonemia in cases of urea cycle defects. Hyperammonemia

⬨Electrolytes should be monitored frequently during loading and maintenance infusions of sodium phenylacetate-sodium benzoate because these medications contain high concentrations of sodium and chloride. Sodium phenylacetate administration may cause potassium depletion.

💎 Clinical pearl ▫Early fluid resuscitation in patients with STEC-HUS is linked to improved short and long-term outcomes ▫Higher hematocrit value at presentation is a predictor of poor outcome and development of oligoanuric HUS Check the following articles for further

▫ The recommended Echo view in evaluation of hemodynamic instability of unknown etiology is “Subcostal 4-chamber”. ▫ The goal is to get quick view and minimize interruptions of chest compression and defibrillation. ▫ Also, you need to minimize the ultrasound gel placed on

☑ Eculizumab, which binds to C5 and inhibits activation of complement pathway, is used in management of atypical HUS. onset of action is within hours. 🎙️Check out our episode about Hemolytic Uremic Syndrome: shorturl.at/fRVDV #PedsICU

📛Norepinephrine should be administered through central line, except in extreme emergency. 🛠️Norepinephrine extravasation is treated with Phentolamine - non-selective alpha blocker.

📛The use of Norepinephrine without adequate intravascular volume, may elevate the blood pressure without improving end organ perfusion. 🛠️Norepinephrine should be administered with repletion of intravascular volume.

○ Vasopressin works independently of the adrenergic receptors. Consider in refractory vasodilatory shock. ○ Learn more about vasopressors with our episode from PICU Doc On Call Bootcamp: podcasts.apple.com/us/podcast/vas… #PedsICU