URx 3 Flashcards
______ is characterized by progressive cyst formation and enlargement in the kidney, as well as other organs such as the spleen, liver, and pancreas, including an increased risk of development of berry aneurysms.
ADPKD is characterized by progressive cyst formation and enlargement in the kidney, as well as other organs such as the spleen, liver, and pancreas.
____ syndrome is characterized by progressive bilateral sensorineural hearing loss with/without ocular defects in a child accompanied by hematuria, proteinuria, and progressive renal insufficiency with a thickened and thinned glomerular basement membrane with “splitting” and “basket weaving” patterns on HPE.
Alport syndrome
In children presenting with aniridia and congenital abnormalities of the kidneys or urinary tract, ____ syndrome must be highly suspected.
WAGR syndrome:
-Wilms tumor,
-Aniridia,
-Genitourinary anomalies, and
-Range of developmental delays
Children with WAGR should be screened for Wilms tumor by abdominal USG every _____ months until the age of 5.
every 3 months until the age of 5.
Other than WAGR syndrome, Wilms tumor may also be a/w _____ conditions/syndromes.
-Beckwith-Wiedemann syndrome (hemihyperplasia),
-macroglossia,
-visceromegaly,
-neurofibromatosis.
_____ typically presents with an erythematous, pruritic, annular lesion with an elevated, soft border that has fine scale and central clearing.
Tinea corporis
Tinea corporis, a dermatophyte infection is usually acquired via ____ routes.
transmitted
-person to person, or
-via fomites), or
-acquired from dogs or cats (Microsporum canis).
The most common sites of involvement in staphylococcal folliculitis are ____ and ____, particularly in young children.
buttocks and thighs
True/False?
Folliculitis often occurs at sites that are occluded, covered by tight clothing, or subject to shaving.
True
What are the differential diagnoses of bacterial folliculitis?
- Acne Vulgaris: papules, pustules, and comedones located mainly on the face and trunk/back; recurrent and chronic
- Hidradenitis Suppurativa: abscesses and sinus tracts located in the axillae, inguinal folds, and anogenital region; chronic condition.
- Keratosis pilaris: follicular papules with a central keratin plug located on the upper outer arms, thighs, and buttocks, and
- Papulopustular rosacea: papules, pustules, and erythema involving the central face.
VERSUS
Bacterial folliculitis: recurring crops of erythematous pustules and papules, usually occurring on sites that are occluded/covered by tight clothing, or subject to shaving; lesions are centered around follicles, and resolve with temporary post-inflammatory hyperpigmentation.
Surgical correction of cryptorchidism should be performed at ____ age.
between 6 and 24 months of age;
Note: spontaneous descent may occur by 4 months of age.
What are the potential complications of cryptorchidism (surgical correction between 6-24 months of age)?
-testicular cancer,
-infertility, or
-inguinal hernia.
Unlike in cryptorchidism, a _____ testis can be manipulated into the scrotum.
retractile testis
In addition to nausea, vomiting, liver injury, and metabolic acidosis, iron toxicity commonly p/w _____ and/or ____ d/t the _____ effect.
hematemesis and/or melena d/t the caustic effects of iron on the gastrointestinal mucosa.
_____ toxicity must be highly suspected in a patient p/w nausea, vomiting, acute hepatotoxicity marked by elevated transaminase enzymes, metabolic acidosis, and mild methemoglobinemia after ingestion of an unknown pill.
acetaminophen toxicity
Approx. ___ % of acetaminophen is metabolized mostly to nontoxic metabolites through glucuronidation, and about ____ % is metabolized through _____ enzymes to the toxic metabolite, N-acetyl-p-benzoquinone imine (NAPQI); NAPQI is further metabolized to non-toxic compounds through _____ process.
~ 90% of acetaminophen is metabolized mostly to nontoxic metabolites through glucuronidation;
~ 5% is metabolized through cytochrome P450 enzymes (specifically CYP2E1) to NAPQI;
NAPQI -> non-toxic compounds through conjugation with Glutathione.
___ is the best initial treatment for acetaminophen overdose, the mechanism of action being _____.
N-acetylcysteine (NAC);
NAC replenishes and increases the glutathione reserves which are then able to conjugate with NAPQI to form nontoxic metabolites.
Acetaminophen toxicity occurs when NAPQI formation (d/t ingestion of large doses) exceeds glutathione conjugation capacity, leading to _____.
covalent binding of the metabolite to cellular proteins, further causing mitochondrial dysfunction and hepatic necrosis.
N-acetylcysteine’s (NAC) efficacy as an antidote to acetaminophen toxicity declines beyond ____ hours after ingestion; hence, a quick diagnosis and treatment is necessary to prevent liver toxicity and failure.
beyond 8 hours after ingestion.
When is NAC specifically indicated in the management of acetaminophen toxicity?
- when acetaminophen concentration is at or above the “possible hepatotoxicity” line on the Rumack-Matthew nomogram in cases of single ingestions at a known time 4 hours or more after ingestion but within 24 hours of ingestion, and
- when serum acetaminophen concentration >10 µg/mL or if transaminase levels indicate hepatocellular injury in cases where ingestion time is unknown or in whom multiple ingestions have taken place.
How is acetaminophen toxicity distinguishable from aspirin or salicylate poisoning, iron poisoning, diabetic ketoacidosis, and hyperosmolar hyperglycemic state, all of which may clinically present similarly?
- Aspirin or salicylate poisoning:
-causes both metabolic acidosis & respiratory alkalosis (d/t tachypnea),
-hyperthermia, and
-tinnitus.
-Sodium bicarbonate used as t/t. - Iron poisoning: causes
-nausea, vomiting, liver toxicity, and
-GI bleeding (hematemesis, melena) d/t caustic GI mucosal injury. - DKA, or Hyperglycemic states: a/w
-high blood glucose levels,
-changes in osmolarity, and
-dehydration plus
-typical s/s: polyuria, polydipsia, and fruity breath smell (in DKA)
-cutoff serum measurement of more than 40 mg/dL in acute poisoning such as in this case (here the salicylate level is 11 mg/dL).
ADPKD is a multisystem progressive disease characterized by bilateral renal cyst formation, kidney enlargement, and extrarenal organ involvement in organs such as the ___ (list all).
liver, pancreas, spleen, cardiac, and arachnoid membranes.
_____ polycystic kidney disease (PKD) is more prevalent, affecting 1 in 400 to 1,000 people, while _____ PKD occurs less frequently, with an estimated prevalence of 1 in 20,000 to 40,000 individuals.
ADPKD is more prevalent;
ARPKD occurs less frequently.
_____ polycystic kidney disease (PKD) usually presents in pre-/peri-natally or early childhood and often causes death in childhood or perinatally.
ARPKD
True/False?
While renal cysts may be detected in childhood or even in utero in ADPKD patients, clinical manifestations typically appear in the third or fourth decade of life.
True.
What is the spectrum of clinical presentation in patients with ADPKD?
While some patients remain asymptomatic their whole lives, about 50-75% of the affected individuals develop ESRD by the age of 70 years.
Data Source: https://www.ncbi.nlm.nih.gov/books/NBK532934
Approx. 85% of patients with ADPKD have _____ mutation (? PKD1, PKD2), whereas 15% have ____ gene mutation which is a/w milder disease, fewer renal cysts, later onset of HTN, and less ESRD as compared to the former.
85% have PKD1 mutation;
15% have PKD2 mutation a/w milder disease, fewer renal cysts, later onset of HTN, and less ESRD.
Liver cysts are a common manifestation of ADPKD and are more prevalent in ____ gender.
more prevalent in females;
*Cysts are shown to increase in size and number in response to high estrogen states (pregnancy, OCP use).
True/False?
Hepatic cysts in patients with ADPKD may forbode progress to significant liver disease or liver failure.
False.
Hepatic cysts may develop but do not progress to significant liver disease or failure.
Citation: https://www.ncbi.nlm.nih.gov/books/NBK532934
_____ is considered a risk factor for the development of HTN in patients with ADPKD, even with normal serum creatinine levels.
A higher cyst burden, as reflected by increased total kidney volume.
Cyst formation in ADPKD most commonly occurs in ______ renal areas.
most common in the distal nephron and collecting ducts;
*can also occur in the proximal and distal tubules.
Hepatic cysts in ____ type of PKD develop early and may even be seen in the prenatal period, and are a/w development of clinically significant liver disease and progress to cirrhosis.
ARPKD
Almost _____ % of patients with ARPKD die early within the first month of life;
however, those who survive the newborn period have a ___ % chance of living up to at least age 20 years, with ESRD developing in about __% of those who reach age 20 years.
20% will die within the first month of life;
those who survive the newborn period have a 90% chance of living to at least age 20 years with ESRD developing in ~ 50% of patients who reach 20 years of age.
Staphylococcal scalded skin syndrome (SSSS) typically presents in children of ___ age, who have commonly had a preceding Staph. aureus infection of _____.
less than 5 years of age;
preceding Staph. aureus infection of the skin, respiratory tract, GI tract, or umbilicus.
True/False?
The cultures of the lesions in Staphylococcal scalded skin syndrome (SSSS) will not show any growth.
True;
since SSSS is a toxin-mediated disease where the exfoliatin toxin that is released by Staph. aureus spreads via hematogenous route to distant sites and targets and splits desmoglein-1in the stratum granulosum of the epidermis.
In children, ______ is the most common cause of nephrotic syndrome.
minimal change disease;
*usually an idiopathic condition that readily responds to prednisone.
Patients with nephrotic syndrome may develop immunosuppression due to ______ reasons.
d/t
-loss of Igs in the urine,
-decreased serum complement, and
-decreased cellular immunity.
_____ is an S-shaped, gram-negative, motile bacillus that is responsible for 72% of gastroenteritis cases in the United States.
Campylobacter jejuni
____gastroenteritis is the second most common cause of bacterial gastroenteritis in the United States after Campylobacter enteritis.
Salmonella gastroenteritis: Lactose fermenting, H₂S producing, motile, gram negative bacilli.
*C. jejuni (most common cause of bacterial GE in the US): Non-lactose fermenting, non-H₂S producing, motile, gram-negative curved rod.
____ in stools is a sign of inflammatory diarrhea.
WBCs
The presence of WBCs on stool microscopy reflecting inflammatory diarrhea narrows down the diagnosis to four common pathogens: ___, _____, ___, and ___.
H₂S producing
1. Salmonella species
Non-H₂S producing
2. Shigella
3. Campylobacter, and
4. Shiga-toxin Producing E. coli (STEC).
MN: Cause Super Slimy/Watery Stool!
Short incubation periods (usually between 2- 6 hours) are characteristic of food poisoning a/w preformed enterotoxins of mainly ______ and _____ bacteria.
Staphylococcus aureus (food exposed to unclean fingers/hands), and
Bacillus cereus (reheated rice).
Longer incubation periods (usually 12-72 hours) are associated with enteric infections involving _____ area of GIT.
intestines
____ is the most appropriate immediate step in the management of a patient p/w difficulty breathing, drooling, hyperextended neck, and “thumb-sign” (enlarged epiglottis protruding from the anterior wall of the hypopharynx) on a lateral x-ray of the neck.
securing the airway by laryngoscopy and intubation.
Urticaria is classified as acute lasting less than ____weeks, and chronic lasting more than _____ weeks.
acute lasting < 6 weeks;
chronic lasting > 6 weeks.
The initial treatment for acute urticaria is/are _____.
nonsedating H1 anti-histaminics (2nd generation) such as Loratadine or Cetirizine.
+/- short-term (5-7 days) oral corticosteroids in patients with severe disease
_____ are the second-line treatment agents in urticaria.
-Sedating (1st generation) H1 antihistamines (eg, diphenhydramine or hydroxyzine),
-H2 antihistamines (eg, cimetidine),
-Leukotriene receptor antagonists (eg, montelukast).
Congenital heart disease is present in up to _____ % of patients with DiGeorge syndrome and includes both cyanotic and acyanotic lesions such as ______.
up to 75% of patients;
-TOF,
-VSD, and
-interrupted aortic arch.
The initial imaging for DiGeorge syndrome includes _____ investigations.
-Echocardiography (at birth) to identify cardiac abnormalities such as VSD, TOF, Truncus Arteriosus, and Pulmonary artery atresia.
-X-ray chest for absent thymic shadow, and
-Renal USG for renal anomalies.
Duodenal atresia typically causes bilious vomiting within ____ duration after birth VERSUS pyloric stenosis which typically p/w immediate, postprandial, projectile nonbilious vomiting, feeding intolerance, weight loss between 2 and 8 weeks of age.
within 24 to 28 hours
A “double bubble” sign on a plain x-ray abdomen is a classic finding in _____, whereas a “double-track” sign may be seen in the setting of _______.
double bubble sign in duodenal atresia;
double-track sign in infantile hypertrophic pyloric stenosis.
_____ investigation p/w ____ finding is critical in making the diagnosis of necrotizing enterocolitis (NEC) which is a life-threatening condition of common occurrence in premature infants p/w feeding intolerance (gastric residuals), abdominal distention, delayed gastric emptying, and bloody stools (+ve guaiac).
Abdominal imaging p/w pneumatosis intestinalis (gas within the bowel wall).
In general, a pre-term or full-term neonate with feeding intolerance should initiate suspicion of ____ conditions.
-Duodenal atresia: vomiting within 24- 48 hours after birth.
-Pyloric stenosis: immediate, postprandial, projectile nonbilious vomiting, usually around 6 weeks old.
-Spontaneous intestinal perforation: abdominal distension with hemodynamic instability.
-Necrotizing enterocolitis (NEC): sudden change in feeding tolerance, abdominal distention, abdominal wall erythema or induration, bloody stools (+ve Guaiac), apnea, lethargy, and s/s of cardiovascular instability.
____ is the most common malignancy of childhood.
Acute lymphocytic leukemia (ALL):
hematologic malignancy of B or T cells characterized by the proliferation of abnormal, immature lymphocytes and their progenitors.
______ therapy is the first-line treatment for apnea of prematurity (AOP), a condition commonly seen in preterm infants (< 37 weeks GA).
Caffeine therapy;
NOTE: Caffeine is a CNS stimulant, that reduces the frequency of apneic episodes by stimulating the respiratory center in the brain.
For severe cases of apnea of prematurity (AOP) ______ can be used to manage obstructive components responsible for the condition.
-nasal continuous positive airway pressure, or
-invasive mechanical ventilation.
Gastroesophageal reflux disease (GERD) in an infant generally presents during the first few months of life, peaking at about ____ months of age, and resolution of symptoms by _____ age.
peaks at about 4 months of age;
resolution of symptoms by 12- 24 months of age.
RSV bronchiolitis, the most common cause of lower respiratory tract infection in infants is typically managed with ___ and ____; hospitalization with ____ treatment is indicated for _____ cases.
fluid resuscitation and supportive care with nebulization;
hospitalization with supplemental O2, bronchodilators, and IV hydration is indicated for
-severe cases with O2 sat. < 90%, or
-patients with h/o prematurity or underlying cardiopulmonary disease.
RSV bronchiolitis typically occurs between ___ and ____ months.
between November and April.
The most common causes of compartment syndrome are ____ (list all).
-Tibial fractures,
-Forearm fractures,
-Knee dislocations,
-Burns, and
-Crush injuries.
What are the “6 P’s” of compartment syndrome?
- Pain out of proportion to findings and passive stretch (first indicator)
- Paresthesias (follows pain)
- Pallor,
- Paralysis,
- Poikilothermia,
- Pulselessness (late finding).
What are the indications for instituting positive pressure ventilation in a neonate at birth based on guidelines of the Neonatal Resuscitation Program (NRP)?
If a child at birth
-has irregular respirations,
-is gasping, or
-has apnea or
-has pulse < 100/min.
The Neonatal Resuscitation Program (NRP) recommends chest compressions in neonates only when the heart rate drops below ___ beats per minute.
60 beats per min.
Normal heart rate at birth is > 100 beats/min.
In people with sickle cell trait (heterozygotes), only ___ % of the Hb is HbS (abnormal), sparing these patients from clinical symptoms most of the time.
35- 40 %
Sickling of the RBCs in the pulmonary vasculature in patients with SCD leads to _____ p/w chest pain and shortness of breath.
Acute chest syndrome p/w chest pain and shortness of breath.
Sickle cell trait is generally benign; complications can arise under extreme conditions such as ___.
high altitude;
complications include
-episodic hematuria,
-an inability to concentrate urine, and -splenic infarcts.
_____ a manifestation of acute rheumatic fever, is a neuropsychiatric disorder characterized by involuntary, brief, random, and irregular movements of the limbs and face, emotional lability, irritability, inappropriate behavior, obsessive-compulsive behavior, and hypotonia.
Sydenhams chorea (SC);
NOTE: most likely d/t “molecular mimicry”, in which anti-GAS antibodies cross-react with the basal ganglia antigens in susceptible hosts.
What are the Jones criteria condition for the diagnosis of acute rheumatic fever?
Diagnosis of acute rheumatic fever requires the presence of
-2 major criteria, or
-1 major + 2 minor criteria, or
-5 minor criteria.
MAJOR Criteria:
-Joints (polyarthritis),
-Carditis,
-Subcutaneous nodules,
-Erythema Marginatum,
-Sydenham chorea
MINOR criteria:
-Fever
-Arthralgia
-Increased ESR
-Increased CRP
-First-degree heart block
_____ is the first-line treatment of Sydenham chorea.
chronic penicillin therapy, initiated with long-acting IM penicillin G benzathine to prevent further GAS infections and thus progression to RHD.
NOTE: SC itself is self-limiting illness.
Chorea-suppressing medications such as Haloperidol (or alternatives such as levetiracetam, valproic acid, and carbamazepine) and/or corticosteroids for immune suppression may be given to ____ cases of Sydenham Chorea (SC).
In patients with significant impairment related to their chorea
Vesicoureteral reflux (VUR) is graded on a scale of 1 to 5, with grades ____ to ____ less likely to resolve spontaneously and more likely to be a/w recurrent febrile UTIs and risk of reflux nephropathy.
grades 4-5 are less likely to resolve spontaneously;
Describe the grading system of vesicoureteral reflux (VUR) based on the severity of the VUR.
Grade 1: reflux fills only the ureter with no ureteric dilation.
Grade 2: reflux fills the ureter and the collecting system without dilation.
Grade 3: reflux fills the ureter and the collecting system with mild dilation of the ureter and no/mild blunting of the calyces.
Grade 4: reflux grossly dilates the ureter and the collecting system with blunting of the calyces and ureteric tortuosity.
Grade 5: massive reflux grossly dilating the collecting system.
_____ caused by ____ pathogens p/w a high fever persisting for 3-5 days with cough and cold symptoms followed by the onset of a pink macular eruption that begins on the trunk and spreads to the neck, face, and extremities within 12-24 hours after resolution of the fever.
Roseola aka Sixth disease, Exanthem Subitum, or Roseola Infantum;
caused by HHV 6 and HHV7.
At 2 months of age, infants should receive _____ vaccines.
MEMORY AID: 6 vaccines at 2 months.
- Hepatitis B (2nd dose)
- Rotavirus,
- DTaP
- H. influenzae type b (HiB),
- Inactivated poliomyelitis (IPV), and
- Pneumococcal conjugate vaccine (PCV).
A term infant not passing meconium within ____ hours, is highly suggestive of the presence of a congenital pathologic process for example a congenital megacolon aka Hirschsprung disease, etc.
48 hours
_____ and _____ are the most common non-suppurative sequela a/w S Pyogenes throat infection.
-polyarthritis seen in ~ 60-80% of patients, and
-carditis seen in ~ 50-70% of patients.
NOTE: both of the above are a/w rheumatic fever
The absence of meconium passage in a patient with trisomy 21 should raise suspicion for _____ associated congenital disorder.
congenital aganglionic megacolon aka Hirschsprung disease.
The first-line treatment agents for Enterobius Vermicularis infection (pinworms) include _____ drugs;
____ is indicated for use in pregnant women.
mebendazole, albendazole, or pyrantel pamoate;
pyrantel pamoate in pregnant women.
Both ____ and _____ are independent risk factors for retinopathy of prematurity (ROP) in pre-term infants under management for NRDS.
prematurity and oxygen therapy
______ test must be done in a neonate with duodenal atresia to assess for commonly associated _____ malformations, especially in those with suspected trisomy 21.
Echocardiogram to assess for cardiac malformations, commonly associated especially in those with suspected trisomy 21.
_____ is the management priority to stabilize a newborn p/w signs and symptoms and a “double bubble” sign on a plain X-ray abdomen indicating duodenal atresia.
gastric decompression with a nasogastric or orogastric tube, and IV fluid replacement (to prepare for surgical correction once the patient is hemodynamically stable).
In a neonate with a “double-bubble” sign (indicating duodenal atresia) and no e/o bowel perforation on a plain radiograph, _____ study must be conducted to rule out ______, as the latter needs immediate/emergency surgical correction.
upper contrast gastrointestinal (GI) study to rule out malrotation;
*malrotation with volvulus would require emergency surgical correction.
What are the clinical consequences of Volkmann ischemic contracture resulting d/t impingement or injury to the brachial artery in a supracondylar # of the humerus?
-shortening of the forearm muscles -fixed elbow flexion,
-forearm pronation,
-wrist flexion, and
-metacarpal-phalangeal joint extension.
see attached image
Identify the tick and the resultant rash in the attached image.
The person bitten by this tick is at risk of what disease?
Ixodes deer tick (Northeast US)
Bite -> r/o Lyme’s disease caused by Borrelia burgdorferi (spirochete).
