3. Vernix Caseosa
• White creamy covering of the
skin especially seen in premature
babies.
• Composed of sebum, fatty acids
and amniotic cells
• Function: lubrication (easy to
pass towards the birth canal) +
bacterial barrier
7. • Damage of C5-C6
• Absence of mono reflex on one side
• Rx: physiotherapy if not recovering within 3
months → Neuroplasty
Erb’s Palsy
• C7-T1 Injury
• Absence of grasp reflex
Klumpke’s Palsy
8. Necrotizing Enterocolitis
Normal
Risk:
• Preterm infants up to 10% in infants <30 weeks
Clinically:
• Abdominal distention
• Residual gastric content
• Bilious vomiting
• Bloody stool
• Abdominal erythema
• Can lead to TCP, DIC and death
Diagnosis:
• X-ray: abdominal distention, thick bowel
walls, pneumoatosis intestinalis, venous portal
gas.
• Pneumoperitoneum suggests perforation
9. Management:
Medical: bowel rest, no oral feeds, gastric decompression, parenteral fluids
and nutrition.
Surgical: laparotomy for pneumoperitoneum, resection of the necrotic bowel
Late complications:
Adhesions and strictures (obstruction), short bowel syndrome resulting in
malabsorption and cholestasis
Necrotizing Enterocolitis
18. Complications of GABHS
•PSGN: antibiotic therapy will NOT prevent this complication.
•Post-streptococcal arthritis: antibiotic therapy will NOT prevent this
complication.
•Rheumatic fever: can be prevented by antibiotics.
•Acute onset of obsessive compulsive symptoms: can be prevented by antibiotics.
22. Otitis Externa
•Caused by: excessive moisture or humidity, cerumen removal.
•Common pathogens: S.aureus and Psudomonas areginosa or Candida albicans.
•It can develop in patients with perforated tympanic membrane secondary to acute otitis
media
•RX: topical antibiotics/corticosteroids… if + AOM = topical + oral
23. Impetigo
•Superficial skin infection involving the upper dermis
•S.aureus is the most common cause + S. pyogens
(GABH)
•RX: topical mupirocin or oral antibiotics such as
dicloxacillin
•Complications: bacteremia, PSGN and Staphylococcus
Scalded Skin Syndrome (SSSS).
26. SSSS
• Caused by: S. aureus species that produce
exofolitative toxins. Patient will present with
fever, tender skin and bullae.
• Large sheets of skin will slough after the illness.
• Nikolsky sign is also present (extension of
bullae when pressure is applied to skin)
• Rx: good wound care and antibiotics IV
27. Scarlet Fever
•Toxin-mediated bacterial illness resulting in a characteristic skin rash caused by GABHS;
producing erythrogenic toxin, transmitted by nasal secretions.
•Clinical features:
• Exanthum. Before or during the exanthum there will be: fever, malaise and exudative
pharyngitis. The exanthum is characterized by:
• Begins on the trunk and moves peripherally
• Skin is erythematous (sandpaper rash)
• Pastia lines (rash on skin creases)
• Desqumation of the skin when infection resolves
•Diagnosis: throat culture for GABHS
•RX: Pencillin VK/ IM benzathine penicillin for penicillin allergic children; erythromycin
IMP to prevent the development of rheumatic fever
•Next slide for pictures.
34. Varicella Zooster
•Transmitted by: respiratory droplets (highly infectious during viral shedding).
•Rash will be first papule, then vesicles, then pustules and finally crusts (within 7 days).
•The rash will start on trunk and then face and extremities. Lesions appearing beyond 10
days indicate defective cellular immunity !
35. Complications of VZ
•Bacterial superinfection (Stap/strep) leading to TSS or necrotizing
fascii, it should be considered if there is new onset of fever.
•Encephalitis/cerebellitis (child will be ataxic ~ will resolve within a
month) or aseptic meningitis.
•In immunocompromised patients it can lead to DIC (mortality rate 20%)
Treatment of VZ
•IV acyclovir for immunocompromised children.
•VZIG is recommended for high risk patients after contact with
chickenpox.
•Vaccination at: 12 months & 3 years
36. Shingles
•Its uncommon in children.
•It is caused by the reactivation.
•Of the latent VZV.
•Most commonly in the thoracic
region but any dermatome can
be affected
•Recurrent shingles suggests:
immunodeficiency (T-cell
function defect)
38. Parvovirus 19
Erythema Infectinosum
Starts with fever, malaise, headache
followed by a slapped cheek a week
later on the face, progressing to a lace-
like rash on the trunk and limbs
41. Begins from behind the ears to the whole body
Complications of measles:
• Pneumonia
• Panencephalitis
• Otitis media
• Encephalomyelitis
Management:
• Vitamin A
• Immunoglobulins for post-exp
• Prophylaxis for high risk groups
There is a post-exposure immunoglobulin given for patients with
immunodeficiency
Measles
42. Mumps
(Paramyovirus)
• The virus gains access to the parotid gland before
dissemination to other tissues.
• Onset of the illness: fever, malaise, parotitis (1st
only one side then both sides).
• Serum amylase levels are often elevated =
indication of pancreatic involvement.
• Hearing loss can follow mumps (unilateral and
transient).
• The disease is mild and self-limiting.
• Infectivity is up to 7 days after parotid swelling.
• Other complications:
• Viral meningitis 10% and encephalitis 1/5k
• Orchitis: uncommon in prepubertal males
CNS involvement occur 10 days following parotitis
43. Rubella
(Togavirus Family)
•Maculopapular rash will start in the face first then the whole body will
fade in 3-5 days.
•Lymphodynopathy; suboccipital and postauricular.
•It’s a mild disease, congenital rubella is a complication in a pregnant
women exposed to rubella
Incubation period is 14-21 days
45. Congenital Rubella
Blueberry muffin babies, jaundice, thrombocytopenia and
hepatosplenomegaly
Characteristic to rubella
• Deafness
• Cataracts
• PDA
46. Roseola Infantum
(Caused by: HHV-6)
Roseola is a disease of children, generally under two years old whose
manifestations are usually limited to a transient rash (exanthem) that
occurs following a fever of about three days' duration.
A 2 year old child with high fever and runny nose of 3 days with nasal
congestion. On the 4th day the fever subsided and a generalized
maculopapular rash appeared
47.
48. Molluscum Contagiosum
• Common in school children with DNA pox virus
• Spread by contact and scratching lesions
• PEARLY papule with central UMBLICUS
• Spontaneous resolution in 6-9 months (can last years)
• Advise to separate Towels and Bath
• Occasional Treated by CRYOTHERAPY
49. Amebiasis
• Entamoeba histolytica, ingestion of the cyst in
contaminated food or water. Symptoms after
1-4 weeks after invading colonic mucosa.
• Most patients are asymptomatic
• Symptomatic: mild colitis to severe dysentery.
• Cramping abdominal pain, tenesmus and
diarrhea (may contain blood), weight loss,
fever. Right shoulder pain and
hepatosplenomegaly.
• It can result in intestinal perforation,
hemorrhage, strictures and a local mass
ameboma.
• Can result in abscess in the liver, brain and
lung.
Ameboma
• Rx: metronidazole + iodoquinol
50. Giardiasis
•A protozoan G.lamblia;feco-oral cyst ingestion
•Clinical features ranges from asymptomatic to explosive diarrhea
•Symptoms occur 1-2 weeks after ingestion and persist up to 6 weeks
•Clinical features: diarrhea (watery and foul smelling), bloating, flatulence
and weight loss
•Diagnosis: direct examination of stool for cyst and trophozoites OR by stool
ELISA
Rx: metronidazole
52. Malaria
RX:
• Chloroquine
• Mefloquine
• Primaquine
• Doxycyline
Most common cause of death is P. falciparm (most severe!)
The most common is P. vivax
Hypnozoite in (Vivax and ovale rx: Primaquine)
54. Kala azar
•In visceral leishmaniasis the RES (liver, spleen and bone marrow) are most
affected resulting in anemia, leukopenia, thromobocytopenia and splenomegaly.
•Clinical features: fever, weakness, weight loss, splenomegaly, black sickness
(hyperpigmentation), (anemia, thrombocytopenia and leukemia) and infections.
Sand fly
55. Leishmaniasis (under microscope)
Laboratory diagnosis:
• Biopsy (spleen, lymph node, bone
marrow) touch preparation.
• Serological (indirect immunoflorecent)
(IgG)
• Skin test (leishmanin antigen) skin test
(–) during active disease but (+) in
recovered patient.
Treatment:
• Amphotericin B & sodium
Stibogluconate
57. Schistosoma
• Liver & spleen can result
in portal hypertension
• Host is a snail that will
penetrate the skin
Mansoni lateral spine
Results in
hepatosplenomegaly
Rx:
• Praziqunatel
58. Enterobius Vermicularis
(Pinworm)
• The most common helminthic
infection. Transmitted feco-orally
(eggs). Usually in preschool or
school children.
• Clinical: anal, vulvular pruritus,
insomonia, anorexia, enuresis
and night-time teeth grinding.
• Rx: single dose of mebendazole
and albendazole and treat all
close contacts
69. Cervical Lymphadenitis
Enlarged tender and inflamed lymph nodes in the cervical area
DDx:
•S. Aureus is the most common bacterial agent
•S. Pyogens
•Mycobacterial infection (TB, atypical mycobacterium)
•B. Henselae (cat scratch disease)
Reactive lymphadenitis: in response to pharynx, teeth and soft tissue
infection of the head and neck
Viral infections: EBV, CMV and HIV
Kawasaki disease (unilateral)
Toxoplasma
Structural lesions of the neck (cysts which can become 2ry infected)
70. Cervical Lymphadenitis
Diagnosis is based on clinical features:
The node will be mobile, tender and warm + enlarged; fever might be
present
Tests are indicated if there was no response to therapy: Tuberculin test,
CBC with ddx and antibody titer for Toxoplasma
If the lymphodynopathy was diffuse and persistent do antibody titer for
CMV, EBV and HIV
RX:
Emperic therapy towards S. pyogens and S. aures which are the most
common cause with 1st G cephalosporins or Methicillin
71. Cystic Hygroma
Occuluded lymphatics usually presenting in the
first 2 years of life
Lateral or midline
Dx: translucent, painless and compressible soft
Rx: surgical excision
75. Cat Scratch Disease
•Caused by: Bartonella henselae
•Most common cause of lymphadinitis > 3 weeks
•Incubation period: 3-30 days
•Present with a red-white papule along the linear scratch + chronic regional lymphadinitis
•Rx: PCR and Stain will show gram (–) bacilli
•The disease is self limiting !!
76. Torticollis
• Most common cause is upper respiratory tract infections,
minor trauma or cervical lymphadenitis
• Serious causes: retropharyngeal abscess & atlantoaxial
subluxation
• Diagnosis: first do a an X-ray to ensure that there is no
cervical spine fracture of dislocation
79. Meningococciemia
• Any organ can be affected by vasculitis or
thromboembolitic disease
• They have a characteristic meningococcal rash
• It can result in: septic shock, DIC, acidosis,
renal and heart failure
• Rx: High dose IV penicillin ASAP !!
86. Voiding Cystourethrogram (VCUG)
Normal Normal
Contrast introduced into the urinary bladder via a urinary catheter then it’s
imaged under fluoroscope during filling of the bladder and during voiding.
88. IgA Nephropathy
• Most common cause of chronic glomerulonephritis.
• Caused by: abnormal clearance of IgA complex
• Clinically: gross hematuria associated with respiratory
infections.
Diagnosis:
• Renal biopsy showing on:
• Light microscope:mesangial matrix proliferation.
• Immunoflurescent: mesangial deposition of IgA
• 50% of patients will have high IgA levels
RX:
• Supportive
• ACE inhibitors
• Steroids and immunosuppressant for patients with
renal insuffiencey
109. Hirschsprung’s Disease
•Congenital aganglionic disease due to the lack of the migration of ganglion from
neural crest cells resulting in the contraction of the distal segment of the colon
and proximal dilation.
•Seen in 1:5000 live births; more in males with 80% of cases having a family
history.
•Clinical features:
• Constipation, vomiting and abdominal distention in the neonatal period
•Diagnosis: rectal biopsy, revealing the absence of ganglionic cells
•RX: resection of the affected segment
111. Intussusception
Clinical features:
• Sudden crampy colicky abdominal pain
• Vomiting and lethargy
• Stool might have blood currant jelly
• On physical exam a sausage shaped mass
may be palpable in the RUQ
Diagnosis:
• Air contrast enema is diagnostic and
therapeutic
Management:
• HYDRATE
• Contrast enema with air
• If contrast enema failed or there is sign of
peritonitis operation is indicated
112. Volvulus & Malrotation
•Bilious vomiting and sudden
onset of abdominal pain in a
healthy infant
•Anorexia and blood tinged stool
Diagnosis:
•Upper intestinal contrast imaging
RX:
•Untwisting and resection
Volvulus
116. Gastroschisis
Gastroschisis is a congenital defect that
involves herniation of abdominal viscera
through the abdominal wall next to the
umbilicus. There is no sac covering the
herniated viscera. It is typically located to the
right of a normally positioned umbilical cord. It
usually presents with polyhydramnios in utero
and is usually detectable on prenatal
ultrasound. The affected infant is typically
delivered spontaneously, preterm, and has
malrotation, stenosis, or atresia of the GI tract.
An oro- or nasogastric tube should be inserted
to decompress the stomach, the airway should
be stabilized, and peripheral intravenous access
should be established for initiation of fluids and
antibiotics. Emergency surgery is necessary to
correct the defect.
125. Atrial Septal Defect
Defect Physical exam Murmur ECG CXR
ASD Increased RV
impulse
Systolic ejection
murmur. mid
and upper left
sternal border.
mid-diastolic
filling rumble
through the
tricuspid valve.
S2 split, high
pulmonary
blood flow
Right axis
deviation
Right ventricular
hypertrophy
right atrial
enlargement
Right ventricle
and atrial
enlargement
with increased
pulmonary
vascular marking
128. Ventricular Septal Defect
Small VSD: harsh holosystolic ejection murmur with thrill in the left
lower sternal border
Moderate VSD: larger shunt CHF might be seen. Holosystolic murmur &
diastolic rumble may be heard at the apex
Large: often casing symptoms of CHF. Systolic murmur is shorter and
lower in pitch. Mitral filling rumble is heard at the apex
Defect Physical exam Murmur ECG CXR
VSD Thrill
High pitched
holosystolic
murmur
Diastolic rumble
at the apex
If small: normal
Moderate: LVH
and RVH if
pulmonary
hypertension is
present
Cardiomegaly
and increased
PVM
If PVR is high
Decreased PVM
129. PDA
Small PDA: no symptoms
Moderate-large: CHF symptoms due to increased
pulmonary blood flow
Defect Physical exam Murmur ECG CXR
VSD Thrill. Brisk pulse
(Slow)
Machinery like
murmur on the
upper left
sternal border
Mild LVH
To LVH and RVH
if pulmonary HT
is present
Cardiomegaly
with increased
PVM
134. Double Aortic Arch
• Can lead to chronic airway obstruction which is not
relieved by corticosteroids or bronchodilators. The
obstruction is relieved by neck extension and worsen
when supine!
137. Erb’s palsy
•Damage of the nerve roots
•C5 and C6 most commonly
•Involving the right arm.
Clinical features:
•Flaccid arm & asymmetric Moro reflex
•Arms are internally rotated
•Forearm is pronated and fingers are flexed.
138. Klumpke’s Palsy
Klumpke’s palsy: less common than erb’s involving C7 and C8
Clinical features: claw hand & horner syndrome (ptosis, miosis and anhydrosis)
144. Choanal Atresia
• Baby will turn cynotic if he tries to breath with nose, when he cry he
will turn pink
• Difficulty in inserting the catheter through the nose
145. Transient Tachypnea of Newborn
• Increased vascular
marking
• Fluids in the lung
fissures
146. Chronic Lung Disease
(Bronchopulmonary Dysplasia)
It’s O2 dependency beyond 28 days of life
Most commonly occur following RDS or other acute lung injury; meconium aspiration syndrome,
mechanical ventilators or infections
This acute injury will result in abnormal healing and scar tissue resulting in fibrosis and remodeling of the
lung parenchyma. The injury will occur by oxidant proteases. Airway compliance will be diminished
resulting in both obstructive and restrictive lung disease.
Clinical features: range from transient oxygen dependency to prolonged need for ventilator support.
Diminished O2, hypercapina on ABG
Episodes of tachypnea, wheezing, respiratory distress
Frequent respiratory infections and PHT
CXR:
Hyperinflated, areas of
atelectasis, cystic
radiodenseties
Other symptoms: increased
caloric need and delayed
growth/development
147. Management of CLD
•Supplemental oxygen
•Bronchiodilators, diuretics
•Optimize caloric intake
•Prevent complications by immunizations and antibiotics
•Prognosis: disease will diminish with time and growth.
However patients will have increased risk of sudden death,
hyperinflation and risk of respiratory infections
148. Foreign Body Aspiration
History of :
• Sudden chock
• Unilateral wheezing
• Unilateral absence of breathing sounds
• Unilateral stridor
Sometimes esophageal foreign bodies can
compress the trachea
176. Brain Tumors
•2nd most common cancer and the leading cause of cancer
death
•Astrocytoma (40%)
•Medulloblastoma (20)
•Ependymoma (8%)
•Brainstem glioma
•Craniopharyngemoa (4%)
Brain tumors are best diagnosed with MRI
178. Langerhans Cell Histocytosis
Collective group of proliferative disorders of dendritic (Langerhans) cells. Presents in a child as
lytic bone lesions A and skin rash or as recurrent otitis media with a mass involving the mastoid
bone. Cells are functionally immature and do not effectively stimulate primary T cells via antigen
presentation.
Cells express S-100 (mesodermal origin) and CD1a. Birbeck granules (“tennis rackets” or rod
shaped on EM) are characteristic B .
179. Neuroblastoma
Malignant tumor of neural crest cells that can arise
anywhere along the sympathetic ganglia and within the
adrenal medulla.
It is the 2nd most common solid tumor (the 1st are brain
tumors)
Peak incidence in the first 5 years of life
75% occur in the abdomen or pelvis.
The effect of the tumor will depend on the location of it. Eg if
it was in the abdomen it will result in abdominal pain, anorexia
and abdominal mass that cross the midline.
The tumor will also have a catecholamine effect which are
produced by it like flushing, HT, headache and sweating
Raccoon eyes
Metastasis:
• Periorbital ecchymosis (periorbital metastasis)
• Skin nodules (Skin metastasis)
• Bone pain (bone metastasis)
• Hepatomegaly
180. Diagnosis:
• Urine excretion of excessive catecholamines including VMA and HVA
• The definitive diagnosis is by a bone marrow biopsy + elevated urine CCA
• CT & MRI for tumor spread
• Bone scan to assesse for metastasis to bone
Neuroblastoma
181. Wilm’s Tumor
•The most common renal tumor in children
•75% of cases before the age of 5
•Associated with Beckwith-Wiedmann syndrome & WAGAR syndrome (Wilm’s tumor,
aniridia, genitourinary abnormalities, mental retardation).
190. Spinal MuscularAtrophy Type-1
Werdng-Hoffman Disease
• Note the bell-shaped chest
• Type 1 occurs before 6 months of age
• Contractures are noted in the left hand
• With inspection of tongue fasciculation's are noted
• Death usually results from respiratory failure by the
age of 1 year
• Type 2 can sit but never walk alone
• Type 3 can walk and might present later in life
Diagnosis
• Gene studies (SMN gene on chromosome 5)
• Muscle biopsy
193. Chiari type-II Malformation
Caused by the down placement of the cerebellum and medulla through the formen magnum blocking
the CSF flow . It is associated with lumbosacral myelomeningocele
Low-normal intelligence and language disorder
197. Spina Bifida Occulta
No neurological defects are present and there is no herniation through the vertebral cleft
198. Meningocele
A midline mass is present overlying the spine. The mass if filled with CSF fluid and can be
transilluminated. Neurological deficits are not present or only very mild.
199. Myelomeningocele
•Most commonly in the lumbosacral region
•It is associated with chiari type II malformation = hydrocephalus.
•Rib anomalies, deformities of the lower extremities
•Genitourinary defects
208. West Syndrome (Infantile Spasms)
• Onset 3-8 months. Rare in >2 years !!
• Etiology: the most common identified cause is tuberous sclerosis other causes
include PKU, HIE, IVH meningitis and encephalitis.
Clinical features:
• Myoclonic jerks lasting 1-2 sec occurring in clusters 5-10 times over 5 minutes
• Jerks are sudden arm extension or head and trunk flexion.
Diagnosis:
• EEG hypsarrhythmia pattern wave occurring in both cerebral hemispheres.
Rx:
• ACTH IM for 4-6 weeks (1st line) / prednisone
• Valporic acid (2nd line)
• Vigabatrin effective for patients with tuberous sclerosis
Prognosis:
• Poor prognosis children will develop moderate to severe mental retardation
211. Benign Rolandic Epilepsy
It’s a nocturnal partial seizures with 2ry generalization
It’s the most common partial seizure epilepsy during childhood. 15% of
epilepsies.
Present at 2-13 years boys>girls. Its AD
Clinical features:
Seizures occur in the early morning hours when patients are asleep with
oral-buccal manifistations (moaning, grunting and pooling of saliva)
Seizures spread to the face and arm then into genrlizated tonic-clonic
Diagnosis:
EEG: spike and sharp wave in the mid-temporal and central regions.
Management: valporic acid or carbamezapine / has excellent prognosis
213. • Slow progressive weakness affecting the legs first
• In DMD children lose the ability to walk by the age of 10 years
• Psudohypertrophy of the calf muscles
• Gower sign
• Waddling gait
• Cardiomyopathies
Diagnosis:
• Clinical
• CK high
• EMG: polyphasic small potential
• Muscle biopsy: lymphocytes infltation, degeneration and lipid deposition
• Absent or decreased dystrophin on immunochemistry
• DNA test will revel gene deletion
Duchenne Muscular Dystrophy
Most common on left side, through posterolateral foramen (85%).
Presents with failure to respond to resuscitation (or respiratory distress).
Mortality high if lungs are hypoplastic.
Rx= After stabilization → surgical repair
Caused by S. aureus as GABHS = cellulitis
Fatal form of deep cellulitis, so the infection extends into the muscle tissue
IV antibiotic is a MUST !!
Periventricular calcification
EBV
Rx: corticosteroids
Typically the disease affects a child between six months and two years of age, and begins with a sudden high fever (39–40 °C; 102.2-104 °F). This can cause, in rare cases, febrile convulsions (also known as febrile seizures or "fever fits") due to the sudden rise in body temperature, but in many cases the child appears normal. After a few days the fever subsides, and just as the child appears to be recovering, a red rash appears. This usually begins on the trunk, spreading to the legs and neck.
Rx ceftrioxone
High morbidity and mortality 5%
It’s a severe reaction to drugs (anti-inflammatory, anticonv, antibiotics)
Resulting in widespread epidermal necrosis and sloughing of the epidermis >30% of skin :’) can result in sepsis, dehydration and death
CUG is done while voiding (the V in VCUG) to see if there is any reflux of the urine back up into the ureters. In your case, IVP or CT scan would probably be the first ordered study for pyelonephritis or hematuria.
Management:
Low dose prophylactic antibiotics to reduce the incidence of UTI
Children with grade 4 or 5 are referred for surgical replantation of the ureters
Inferior pole of the kidney fuse abnormally. It will be trapped under the inferior mesenteric artery. Kidney function is normal but associated with hydronephrosis, renal stones and increased risk of infection, it’s associated with chromosomal syndromes (Turner, 13,18 and 21)
Most common cause of renal mass in newborns !!
Sporadic
Autosomal recessive: “in pediatrics”
They have maternal history of oligohydroamnios > pulmonary hypoplasia
Greatly enlarged cystic kidney
Severe hypertension !!
Liver involvement in some cases it is progressive and require renal transplantation !
Retinoblastoma (AUTOSOMAL DOMINANT)
It is the most common childhood ocular malignancy
Present between the age of 3-18 months, most cases are diagnosed before the age of 5 years.
Etiology:
Deletion of the growth suppressor gene on both allels of the 13q. it requires two deletion or mutation (two-hit mutation), mutations can be sporadic
Clinical features:
Leukocoria
Strabismus
Glaucoma, hemorrhage, retinal detachment,
The HALLMARK OF THE DISEASE (Calcification within the tumor)
Diagnosis:
Visual inspection with opthalmoscope
Ocular ultrasound or CT
Management:
Early diagnosis is very important, it MUST be suspected in any patient with leukocoria.
Large tumors: poor prognosis Rx: remove the eye
Small tumors: treated with external beam radiation
Very small: cryotherapy or laser
Outcome is excellent if it was identified earlier, cure rate is up to 90% if the tumor did not extend beyond the sclera or into the orbit.
Neonatal conjunctivitis can be caused by N.gonorrhea or C.T / Herpes simplex Or S. aureus/S. pnuemonia etc
Gonorrhea is treated with IV ceftrioxone + eye saline
If the onset was between 2-5 days (Gonorrhea)
Onset >5 days (Chlamydia)
double-bubble sign (air bubbles in the stomach and duodenum) is diagnostic of duodenal atresia, a condition classically associated with Down’s syndrome. Duodenal atresia can cause constipation, but that is usually after meconium has been passed. Duodenal atresia presents clinically with bilious emesis within the first few hours of life. Physical examination is notable for abdominal distension of predominantly the upper abdomen.
Barium enema
A volvulus with mal- rotation is the most critical cause of bowel obstruction. It presents in the first week of life with bilious emesis and constipation. An upper GI series with an absent or abnormal position of the ligament of Treitz confirms the diagnosis of malrotation.
A Meckel’s diverticu-lum is a vestigial remnant of the embryonic yolk sac, typically seen as an outpouching of the ileum. Intussusception may also compli-cate Meckel’s diverticulum; however, these di- verticuli typically manifest with painless rectal bleeding within the first 2 years of life, second-ary to mucosal ulceration of ectopic gastric tissue within the diverticulum. Intussusception associated with Meckel’s is often seen in older boys.
Tracheoesophageal fistula (TEF) is a relatively common congenital anomaly involving the respiratory tract. It occurs in approximately 1 in 4000 live births. TEF occurs with esophageal atresia (EA) in >90% of cases. In the most common form of TEF and EA, the esophagus ends in a blind pouch, and the TEF is connected to the distal esophagus. TEF and EA result from a defect in lateral septation of the foregut into the esophagus and trachea. TEF and EA often occur with the VACTERL (Vertebral, Anorectal, Cardio- vascular, Tracheal, Esophageal, Renal, and Limb abnormalities) syndrome, as seen in this patient with the holosystolic murmur of a ventricular septal defect and limb abnormalities. In cases with EA, there is polyhydramnios in utero. These babies present with excessive secretions that cause frothing at the mouth and nose, drooling, choking, cyanosis, and respiratory distress immediately after birth. If the trachea is connected to the distal esophagus, gastric distention can occur. The symptoms of respiratory distress are exacerbated by feeding. In the presence of EA, a naso- or orogastric tube can be passed only 10–15 cm down the airway and cannot be passed into the stomach. As shown in the image, the tube coils in the upper esophageal pouch. With a distal TEF, a distended gas-filled stomach is seen on x-ray of the chest, and the TEF may be seen on a lateral view. Fluoroscopy can confirm the diagnosis of EA.
Omphalocele is a congenital defect in which there is herniation of abdominal viscera through the abdominal wall into the base of the umbilical cord. Un-like an umbilical hernia, the viscera herniate into a sac that is covered by amniotic mem-brane and peritoneum but no skin. Omphalocele typically presents with polyhydramnios in utero, and the infant is often born prematurely and has other GI and cardiac defects. Ten percent of infants with omphalocele have Beckwith- Wiedemann syndrome (exophthalmos, macro-glossia, gigantism, hyperinsulinemia, and hypoglycemia). The exposed intestine should be wrapped sterilely to minimize insensible fluid losses and loss of heat. An oroor nasogastric tube should be inserted to decompress the stomach, the airway should be stabilized, and peripheral intravenous access should be established for initiation of fluids and antibiotics. The omphalocele is emergently repaired with surgery.
CF is a fatal autosomal recessive disease that is more common in white people. CF may have respiratory, GI, sinus, and/or musculoskeletal manifestations and can cause infertility. Pancreatic insufficiency affects 85% of patients with CF, and leads to steatorrhea with the passage of bulky, foul-smelling, floating stools. This thick stool can act as a lead point for intussusception in patients with CF. Positive sweat test is the definitive way to diagnose CF. Delayed passage of meconium for >48 hours can be an initial sign of CF, but there are no dilated or narrowed loops of bowel in patients with CF. It is important to know that most patients (50%) usually present with failure to thrive or respiratory compromise. Additionally, CF is most prevalent in white people.
HSP is a systemic vasculitic syndrome characterized by the deposition of IgA-containing immune complexes in tissues. There is a classic tetrad of rash (palpa- ble purpura), arthralgias, abdominal pain (of- ten colicky, associated with emesis), and renal disease that can manifest over days to several weeks in any order. Intussusception is a rare complication of HSP that may result from intestinal hemorrhage and hematoma formation serving as a lead point.
Hemophilia A is an X-linked recessive coagulopathy involving a deficiency in coagulation factor VIII that may lead to mild to severe bleeding, depending on the factor activity. A prolonged activated partial thromboplastin time is seen with hemophilia. Prothrombin time, bleeding time, and platelet count are expected to be normal in a patient with hemophilia.
Hemophilia B (Christ- mas disease) is an X-linked recessive coagulopathy involving a deficiency of coagulation factor IX that may lead to mild to severe bleeding, depending on the factor activity. A prolonged activated partial thromboplastin time is seen with hemophilia. Prothrombin time, bleeding time, and platelet count are expected to be normal in a patient with hemophilia.
Management: closure by open heart surgery to prevent right sided heart failure, pulmonary hypertension and embolism.
ASDs are often as-ymptomatic and cause acyanotic heart disease. As described in this patient, physical examina-tion may be remarkable for a wide, fixed, split S2 with a systolic ejection murmur at the left upper sternal border. Some patients also have a mid-diastolic rumble at the left lower sternal border. Both murmurs represent increased blood flow across the pulmonic and tricuspid valves.
Management:
Medical management for CHF
Large VSD are associated with pulmonary infections
Surgical closure is recommended for
1- Heart failure
2- Large VSD with pulmonary hypertension at 3-6 months
3- Mild-moderate VSD closed at 2 and 6 years
PDA causes acyanotic congenital heart disease. PDAs account for 10% of congenital heart disease, occurring with a high incidence in preterm infants and a 2:1 female:male predominance. Small PDAs are asymptomatic, while large ones may cause CHF, failure to thrive, and recurrent lower respiratory tract infections. On physical examination, a continuous machinery murmur heard best at the left upper sternal border, and bounding peripheral pulses may be present. There may also be a prominent apical impulse and a thrill. X-ray of the chest may show cardiomegaly and increased pulmonary vascular markings. PDAs usually close within the first month of life. In preterm infants, indomethacin may be administered to close the PDA. PDAs may also be surgically ligated or coil embolized if necessary.
Rx: indomethacin in premature infants
Surgical closure by coil embolization
Tetralogy of Fallot (TOF) is the third most common cause of cy-anotic heart disease in neonates, after D-TGA and hypoplastic left heart syndrome. It is com- posed of four defects: right ventricular outflow tract obstruction, VSD, right ventricular hypertrophy, and an overriding aorta. TOF usually presents in infancy with cyanotic spells and agitation. The spells either resolve spontaneously or can lead to hypoxia, metabolic acidosis, and death. On physical examination, these infants have a normal S1, single S2, and a harsh systolic crescendo-decrescendo ejection murmur (due to right ventricular outflow tract obstruction), which is loudest over the left up- per sternal border and radiates to the back. A boot-shaped heart may be seen on x-ray of the chest and there are increased pulmonary vascular markings. TOF is corrected surgically.
Coarctation of the aorta is a cause of acyanotic heart disease. It accounts for 8% of congenital heart defects, with a 2:1 male:female predominance. Coarctation of the aorta may be associated with a bicuspid aortic valve in 80% of cases. CHF may develop in infancy in approximately 10% of patients. It manifests as a systolic ejection murmur at the left upper sternal border, radiating to the interscapular region. Physical examination is also remarkable for weak and delayed femoral pulses relative to the upper extremity pulses, and hypertension in the upper extremities. A “reverse 3” sign may be seen on x-ray of the chest due to constriction of the aorta at the coarctation and dilation of the aorta pre- and postcoarctation. Notching of the ribs may also be seen due to collateral circulation through the intercostal arteries, and erosion of the ribs by the collaterals. The defect may be repaired via angioplasty or surgery.
Dextraposed transpo-sition of the great arteries (D-TGA) is the most common cause of cyanotic heart disease in neo-nates. It accounts for 5% of congenital heart defects. With this defect, the aorta arises from the right ventricle and the pulmonary artery arises from the LV. This leads to pulmonary and systemic circuits that are in parallel as opposed to in series. The deoxygenated blood is therefore recirculated through the body in the systemic circulation, while the oxygenated blood only flows through the pulmonary circulation. A lesion, such as an atrial septal defect (ASD), ventricular septal defect (VSD), or pa- tent ductus arteriosus (PDA), is therefore required for mixing of the systemic and pulmonary circulations for survival. D-TGA usually presents at birth with cyanosis and tachypnea. Plain film radiographs demonstrate an egg-shaped silhouette due to the absent main pulmonary artery stem and small heart base. Prostaglandin E1 is used to keep the PDA open and increase mixing of deoxygenated and oxygenated blood. Balloon atrial septostomy can also be used if necessary. An arterial switch surgical procedure is used to repair the defect.
Transient tachypnea of newborn:
It is a self-limited condition characterized by tachypnea >50, mild retractions, hypoxia and grunting usually without signs of severe respiratory depression. Cyanosis is not usually seen but if give O2 of 30-40%.
Its seen in
Large premature infants
Term infants born by C/S
Infants of diabetic mothers
Its caused by retained lung fluids or slow resorption of lung fluids
CXR: prominent vascular marking and lung fissures and small pleural effusion
If air bronchogram is seen this suggests RDS
Rx: erythromycin/azithromycin
Afebrile pneumonia
Autosomal recessive, results in decreased production of pyruvate kinase isoenzyme which will result in ATP deletion and reduced RBC survival
Clinical: jaundice, pallor, splenomegaly and kernicterus !! more common in Asians
Laboratory: anemia, blood smear will show polychromatic RBC
Diagnosis: decrease PK in RBC
RX: transfusion and splenectomy
Accumulation of fluid in at least 2 fetal compartments
Can be caused by Rh- incompatibility, Noonan syndrome, syphilis,
Patients will present with
Abdominal mass (most common presentation)
Abdominal pain in 50%, hematuria 25%, HT and (fever, weight loss, anorexia)
Prognosis excellent 95% cure !
AD; chromosome 19. Trinucleotide repeat disorder.
Increased head circumference >97th percentile
Large anterior fontanelle and split sutures
Sunset sign, downward deviation of both eyes caused by pressure of the 3rd ventricle
Older children with hydrocephalus:
1-vomiting and nausea 2-headaches 3-6th nerve palsy 4-papilledema 5-downward Babnski
Does not require treatment. Excellent prognosis
Requires surgery. Excellent prognosis
Requires urgent surgical repair within 24 hours of birth
90% survive to adolescence but many are handicapped !!
Wheelchair dependency, bladder and bowel incontinence, mental retardation, seizures, pressure sores and fractures
Hypsarrhythmia
X-linked disorder characterized by myofiber degenration
Onset of symptoms 2-5 years
Caused by the deletion of the dystrophin gene on chromosome X
DMD mean age is 5.5 years
Hereditary degeneration of the peripheral nerve, initially the peroneal nerve
AR chromosome 13. Caused by reduced synthesis of copper binding protein and defective excretion of copper in bile leading to the accumulation of copper in the liver, brain, kidney and cornea. This will result in liver cirrhosis, hepatitis and portal hypertension. Renal tubular dysfunction with Vitamin-D resistant rickets. Urine copper in increased
Definitive: liver biopsy high copper OR genetic mutation
RX: pencillinamine or trientien both will promote urinary copper excretion !
Zinc will reduce copper absorption
