Pediatric MCQ and Nots

Q. Patients with nephrotic syndrome: A. Have high serum albumin levels. B. Are likely to be hypertensive. C. Need a renal biopsy to define cause. D. Should have pneumococcal vaccination when in remission. E. Stay on life-long penicillin prophylaxis

B ... #Sickle cell disease SCD is the most common cause of pediatric stroke. - Children with the most severe and prevalent sickle type, homozygous S disease (HbSS), have the highest risk.5 SCD brain vasculopathy causes both overt stroke and “silent” cerebral infarcts, affecting neurological and cognitive function. - Prior to systematic screening and intervention, HbSS-associated pediatric stroke prevalence in the United States and France was 11%. - Highest risk of first overt ischemic stroke is within the first decade of life. - Strokes continue to accumulate through childhood. - Consequences of pediatric SCD stroke include mortality and long-term impaired function and development. - SCD cerebral vasculopathy is mediated by defects in vascular hemodynamics, hemolysis-associated oxidative stress, hemostatic activation, and cellular adhesion. - Pediatric SCD stroke often follows within days of acute inflammatory disease manifestations such as pain crises and acute chest syndrome. - Low steady-state hemoglobin and acute severe anemia have also been shown to increase stroke risk in patients with SCD and certain genetic variants

Cystic hygroma This commonly arises in the neck as a fluid-filled lesion of lymphatic origin. It can found anywhere there is lymphatic drainage, including axilla,abdominal wall and trunk. It may present at birth or within the first 2 years of life. Surgical excision should be performed early. Aspiration of the cysts and injection of the antitumour agent OK432 is a new treatment.

"Blue dot" sign, torsion of the appendix of testis and appendix of epididymis.

Bohn's nodules Are smooth whitish bumps or cysts which are sometimes found in the mouths of newborns. They are found at the junction of the hard and soft palate, and along lingual and buccal parts of the dental ridges, away from the midline. These nodules are 1-3 mm in size, and filled with keratin. The nodes are a result of cystic degeneration of epithelial rests of the dental lamina (rests of Serres). They are benign, and usually disappear within the first three months of life. Bohn's nodules are similar in appearance to Epstein's pearls, which develop on the roof of the mouth. Thanks for all

“Red man syndrome” Often associated with a rapid infusion of the first dose of Vancomycin over < 60 min. Initially attributed to impurities found in vancomycin preparations, but vancomycin with impurities removed continue to have Red Man syndrome. Other antibiotics have also induced Red man syndrome, including ciprofloxacin, amphotericin B, rifampicin and teicoplanin

This rash appeared after the use of an antibiotic. What is the diagnosis & which antibiotic causes this condition?

Tinea Faciei

No significant history. No local or systemic symptoms apart from a mildly pruritic skin lesion. No other physical findings. There is a kitten at home. Diagnosis ?

NEC: Pneumatosis intestinalis with gas under diaphragm (perforation)

Given the incubation period for rabies, PEP is a medical urgency, not emergency. 🏑 recommendation that rabies PEP be considered after any physical contact with bats and when a bat is found in the same room. 🔔Rabies PEP is most effective when applied expeditiously. 🔔should be initiated in the asymptomatic person as soon as possible. 🎳The vaccine and RIG 🙆‍♀️ contraindicated once symptoms develop. 🥈 passive immunization with RIG. ✔️ dose of 20IU/kg ✔️As much of the dose is infused around the wound as possible ✔️ The remainder is injected intramuscularly in a limb distant from the one injected with the killed vaccine. 🔔Like other immunoglobulin preparations, RIG interferes with the take of live viral vaccines for at least 4 mo after administration of the RIG dose. 🥉inactivated vaccine. ✔️ intramuscularly in a 1-mL volume in the deltoid or anterolateral thigh. ✔️ days 0, 3, 7, and 14 after presentation. 🤨Injection into the gluteal area is associated with a blunted antibody response, so this area should not be used. Safe during pregnancy S/E : Pain and erythema local adenopathy, headache myalgias, allergy Preexposure Prophylaxis 🔔The killed rabies vaccine can be given to prevent rabies in persons at high risk for exposure to wild-type virus, including laboratory personnel working with rabies virus, veterinarians 🔔 days 0, 7, and 21 or 28. NELSON 21

rabies virus Theoretically, rabies can infect any mammal but true animal reservoirs that are limited to terrestrial carnivores and bats. 🐕Worldwide, transmission from dogs accounts for > 90% of human cases 🌎 Africa and Asia,prominent reservoirs➡️ jackals, mongooses, and raccoon, dogs. 🐿Rabies is rare in small mammals, including mice, squirrels, and rabbits 🤓to date, no animal-to- human transmission from these animals has been documented. 🦇often bat-associated human rabies there was no history of a bat bite. 😵There have been five outbreaks of rabies associated with solid-organ &corneal transplantations. Transmission 👀virus is found in large quantities in the saliva of infected animals ➡️ almost exclusively through inoculation of the infected saliva. increased with multiple bites and if the inoculation occurs in highly innervated parts of the body such as the face and the hands. 👀Infection does not occur after exposure of intact skin to infected secretions virus may enter the body through intact mucous membranes. 👀No case of nosocomial transmission has been documented but caregivers of a patient with rabies are advised to use full barrier precautions. -rapidly inactivated in the environment, and contamination of fomites is not a mechanism of spread. -Rapid dissemination occurs throughout the brain and spinal cord before symptoms appear. 🔔The pathologic hallmark ➡️ the Negri bodies ➡️ clumped viral nucleocapsids. Negri bodies can be absent in documented rabies 🤢 Rabies leads to tetrahydrobiopterin deficiency in human rabies causes severe deficiencies in dopamine, norepinephrine, and serotonin metabolism. -Many victims of rabies die from uncontrolled cardiac dysrhythmia. Clinical Manifestations 😵 incubation period for rabies is 1-3 mo. 🤕 In severe wounds to the head➡️ within 5 days after exposure 😲incubation period can extend to 8 yr. 🦇Types🦇 🥇Encephalitic or furious rabies: 🎤 fever, sore throat, malaise, headache, nausea and vomiting 🎤 weakness,paresthesia and pruritus at or near the site of the bite that then extend along the affected limb. 🎤agitation, sleep disturbance, or depressed mentation 🎤 periods of lucidity alternating with periods of profound encephalopathy. 🎤Hydrophobia and aerophobia are the cardinal signs 😢The illness is progressive. 😢Death almost always occurs within 1-2 days of hospitalization 🥈 as paralytic or dumb rabies is seen much 🎗less frequently 🎗fevers and ascending motor weakness affecting both the limbs and the cranial nerves. 🎗 some element of encephalopathy. 🎗progresses subacutely. 🔍Differential Diagnosis🔍 🏑cerebral infections, tetanus, and intoxications 🏑autoimmune encephalitis, other infectious forms of encephalitis, 🏑 psychiatric illness 🏑drug abuse 🏑 conversion disorders. 🔔 Paralytic rabies is frequently confused with Guillain-Barré syndrome. 🔍Diagnosis🔍 🎤Reverse transcription polymerase chain reaction is the most sensitive available assay. 🎤MRI findings in the brain are late. 🎗Treatment and Prognosis🎗 💔Rabies is generally fatal. 💔Neither rabies immunoglobulin (RIG) nor rabies vaccine provides benefit once symptoms have appeared. ✔️ vaccination of domestic animals ✔️ education to avoid wild animals, stray animals, and animals with unusual behavior. 🥇The first step in rabies PEP ((Postexposure prophylaxis )) ➡️ cleanse the wound 🎗Soapy water:sufficient to inactivate an enveloped virus. 🎗iodine-containing preparations, are virucidal and should be used in addition to soap ❌Primary closure is avoided ✔️Antibiotics and tetanus prophylaxis should be applied According to wound care criteria. 🔔Postexposure Prophylaxis🔔 🥊During the 10-day observation period, at the first sign of rabies in the biting dog, cat, or ferret, treatment of the exposed person with RIG (human) and vaccine should be initiated. 🥊The animal should be euthanized immediately and tested. † The animal should be euthanized and tested as soon as possible. 🥊Immunization is discontinued if the immunofluorescent test result for the animal is negative. 🔍RIG, rabies immunoglobulin.

PREAURICULAR SINUSES AND PITS preauricular sinus is a common congenital malformation characterized by a nodule, dent or dimple located anywhere adjacent to the external ear ▶️Unilateral or bilateral. ▶️May be Familial & common among females. -Most preauricular sinuses are asymptomatic, and remain untreated unless they become infected. -Association : ▶️((branchiootorenal dysplasia 1 syndrome))A.D ▶️Ext ear malform, branchial fistulas, deafness & renal anomaly. Investigation - ultrasonography for exclude renal anomaly. -If chronically infected➡️ retention cysts may form and drain intermittently TTT :may require excision SKIN DIMPLES -Def:Cutaneous depressions over bony prominences in the acral area. -Associated: pits and creases. ▶️May occur in normal children. ▶️May association with dysmorphologic syndromes. -Causes: as a result of interposition of tissue between a sharp bony point and the uterine wall➡️ leads to decreased subcutaneous tissue formation. -Dimples may also be present overlying an area of bone hypoplasia. -Bilateral acromial skin dimples are usually an isolated finding, but they are also seen in association with deletion of the long arm of ((chromosome 18)) . -Occur over the patella in ((congenital rubella)) . -Occur over the lateral aspects of the knees and elbows in ((prune-belly syndrome)) . -Occur over pretibial surface in ((campomelic dwarfs)) . -Occur as shape of an H on the chin in ((whistling-face syndrome)) . SACRAL DIMPLE -Common and usually are isolated findings. -Seen in multiple syndromes or in association with spina bifida occulta and diastomyelia. Investigation - Ultrasonography during the ((1st 3 mo)) before ossification of the posterior elements of the lower spine➡️ in infants with an isolated sacral dimple. -MRI of the spine is indicated if there is a strong suspicion of a spinal dysraphism➡️look to our photos ((NELSON))