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Gynecomastia is defined as the visible or palpable development of breast
tissue in boys or men. It has been divided into four types: Type I: (pubertal or
benign adolescent breast hypertrophy) refers to the common entity seen in
pubertal males. Incidence may be as high as 60-70%. It is typically a firm,
tender, subareolar mass anywhere from 1-5 cm in diameter. The pubertal
adolescent frequently complains of pain in the breasts, particularly when
wearing binding clothing. It usually spontaneously resolves within 2 years.
Type II (physiological gynecomastia without evidence of underlying disease,
or with evidence of organic disease including the effects of specific drugs)
refers to a generalized, nonpainful breast enlargement. It is essential to
differentiate between physiologic gynecomastia and breast enlargement due either
to a pathologic process or to the use of a specific drug. Careful history taking
regarding the time of onset, family history, duration of enlargement, history of
systemic illness, weight change, and drug or medication use, is important.
Physical examination should include height, weight, blood pressure, breast size,
and Tanner staging of both breasts and genitals, in addition to a neurologic
assessment.
Type III gynecomastia is general obesity simulating gynecomastia, and Type IV
is pectoral muscle hypertrophy.
COMMON CAUSES OF TYPE II GYNECOMASTIA I.
Idiopathic
II. Familial causes a. Associated with anosmia and
testicular hypertrophy. b. Reifenstein’s syndrome (male
pseudohermaphroditism 2ry to partial androgen insensitivity) c. Associated
with hypogonadism and small penis
III. Specific illnesses or syndromes
a. Kleinfelter b. Male pseudohermaphroditism c. Testicular
feminization syndrome d. Tumors e. Leukemia f. Hemophilia g.
Leprosy h. Chronic glomerulonephritis
IV. Miscellaneous drugs a.
amphetamines b. anabolic steroids c. birth control pills d.
cimetidine e. diazepam f. corticosteroids g. digitalis h.
estrogens j. human chorionic gonadotropin k. insulin l. isoniazid and
other TB drugs m. ketoconazole n. marijuana o. methadone and other
narcotics p. reserpine q. tricyclic antidepressants
Ovarian cysts in fetus and infants are usually follicular in nature and less
than 2 cm in size. They are commonly diagnosed between the 28th and 39th wk. of
gestation by sonography. Hypotheses on etiology are:
(1) Excessive fetal
gonadotropic activity,
(2) enzymatic abnormalities of the theca interna, and
(3)
abnormal stimulation by the mother HCG. Obstetric management consists on
observation and vaginal delivery.
After birth, diagnostic assessment and
management will depend on the size and sonographic characteristics of the cyst.
Simple anechoic cysts, and those less than 5 cm in size can be observed for
spontaneous resolution.
Cyst with fluid debris, clot, septated or solid (complex
nature), and larger than 5 cm should undergo surgical excision due to the higher
incidence of torsion, perforation and hemorrhage associated to them.
Percutaneous aspiration of large simple cysts with follow-up sonography is a
well-accepted therapy, preserving surgery for recurrent or complicated cases.
Surgical therapy is either cystectomy or oophorectomy that can result in loss of
normal ovarian tissue.
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What entities are associated with absent radii? VATER syndrome
(anomalies of vertebrae, anus {imperforate}, tracheosophageal region and radii);
Fanconi’s anemia; congenital thrombocytopenia (TAR syndrome); and Holt-Oram
syndrome (associated with a secundum ASD).
What is Sprengel’s
deformity? Congenital elevation of the scapula is a failure of scapular
descent during fetal life, resulting is an elevated, hypoplastic scapula. The
affected side of the neck is shorter and fuller and gives the appearance of
torticollis. It is associated with congenital scoliosis and renal anomalies.
What is the pathophysiology of Osgood-Schlatter disease?
The entity consists of painful swelling of one or both tibial tubercles
at the insertion of the patellar tendon. The disease is very common in
adolescents, usually beginning between the ages of 11 and 15. Vigorous exercise
results in stressful pulling of the patellar tendon. Although the tubercles do
enlarge from this repetitive strain, the primary problem appears to be a
low-grade tendinitis and subsequent new heterophil bone formation in the distal
tendon itself. Ossicles have been found in the tendon separate from the tubercle
and their removal provides relief. Fortunately, this entity rarely requires
surgery. Restriction of activity for two to three weeks and gradual resumption
of activity usually diminishes the symptoms.
What are the
osteochondroses? They are a group of disorders in which degeneration
and aseptic necrosis of unclear cause involve the ossification or growth
centers, recalcification then occurring. The patient usually presents with pain
in the affected area. The commonly affected sites include: tarsonavicular bone,
capitellum of the elbow, carpal lunate, distal ulnar epiphysis, head of femur,
head of second metatarsal, Kohler’s disease, Panner’s disease,
Legg-Calvé-Perthes disease, Burn’s disease etc.
What are the
pathologic stages in Legg-Calvé-Perthes (LCP) disease? LCP is a
condition of aseptic necrosis of the femoral head involving children primarily
ages 4-10. The firs phase is the incipient or synovitis stage; lasting 1-3
weeks, this stage is characterized by an increase in hip-joint fluid and a
swollen synovium associated with reduced movement. the second stage is avascular
necrosis; lasting 6 months to 1 year, the blood supply stops to part (or all) of
the head of the femur. That portion of the bone essentially dies, but the
contour of the femoral head remains unchanged. The third and longest stage is
fragmentation or regeneration and revascularization; lasting 1-3 years, the
blood supply returns and causes both resorption of necrotic bone and laying down
of new immature bone. Permanent hip deformity can occur in this stage. It is
important to note that plain radiographs may lag behind the progression of the
disorder by as much as 3-6 months. Radionuclide bone scans are much better
because early ischemia and avascular necrosis are depicted as decreased
localization's of isotope.
What are the characteristic x-ray
changes, clinical findings, and history in a child with an osteoid osteoma?
Osteoid osteoma is typically seen in older children and adolescents and
exhibits a male predominance. Most children complain of localized pain usually
in the femur and tibia; however, arms and vertebrae may also be involved.
Radiographs and CT scans demonstrate an “osteolytic area surrounded by densely
sclerotic reactive bone”. Bone scans reveal “hot spots”. The site is usually
less than 1 cm in diameter and arises at the junction of old and new cortex.
Pathologically the lesion is highly vascularized fibrous tissue with an osteoid
matrix and poorly calcified bone spicules surrounded by a dense zone of
sclerotic bone.
What should be noted on physical examination in a
child with a suspected fracture? Assess the “five P’s” in the affected
extremity; Pain and point tenderness, Pulse (distal to the fracture), Pallor,
Paresthesia (distal to the fracture), and Paralysis (distal to the fracture).
The involved extremity should also be carefully examined for deformity,
swelling, crepitus, discoloration, and open wounds. A primary concern in any
evaluation is a distal neurovascular compromise, which may require immediate
surgical intervention.
What are the indications for open
reduction of a fracture? An open reduction is an operative reduction in
which pins are fixed into the separate fragments to promote proximity for
healing. Indications include 1) failed closed reduction (often in children with
displaced forearm, tibial, or femoral fractures; 2) displaced intra-articular
fractures; 3) displaced Salter-Harris III and IV fractures (to prevent premature
growth plate closure) and 4) patients with head trauma.
What is
the difference between subluxation and dislocation? Subluxation is an
incomplete or partial dislocation.
What is metatarsus adductus?
A kidney-shaped foot with the front part of the foot (forefoot) turned
inward. It is believed to result from intrauterine constriction. Most cases are
mild and flexible, with the foot easily dorsiflexed and the lateral aspect
easily straightened by passive stretching. Improvement usually occurs within two
months with passive stretching exercises. Rigid, more fixed deformities may
require casting.
source : Pediatric Pearls -- Orthopedics
Newer Antiepileptic Drugs Since 1994, three new antiepileptic drugs (AEDs) have been introduced.
Additionally, drugs under investigation are in rapid progress. We will review
them briefly.
FELBAMATE. Released by Wallace pharmaceuticals in 1993 as
Felbatol®, felbamate was the first new AED to be released in the US since the
early 1970s. Although it appeared to be an effective, broad range AED,
significant untoward side effects (aplastic anemia) became apparent with general
release and its use has fallen out of favor. It remains a drug to be considered
for use with refractory generalized (specifically Lenox-Gastaut syndrome) or
focal seizures. In children dose ranges from 15-45 mg/kg BID or TID, some
children tolerating and improving on larger doses. Serum concentration are not
correlated with efficacy. Most common side effects include insomnia, anorexia,
and weight loss; others include dizziness, nausea, vomiting, diplopia and
headache. Two of these, anorexia and insomnia, can be minimized by increasing
the dose slowly at 2 weeks intervals and by giving it after meals and at
bedtime. The drug has significant interactions with carbamazepine decreasing
serum concentration but increasing the epoxide metabolite, leading to clinical
toxicity. Felbamate increases the serum concentration of valproate and phenytoin
by reducing its clearance. As mentioned, about one year after its release, a
number of patients developed aplastic anemia, and hepatic toxicity. As of July
1995, 31 patients were reported, 10 of whom 10 died. At present, it is
recommended that children taking this drug be followed up closely with weekly
liver function test and complete blood counts.
GABAPENTIN. GBP is produced by Parke-Davis and is released
as Neurontin®. It is labeled for adjunctive therapy in the treatment of complex
partial seizures with or without generalization in individuals 12 years and
older. Little has been published with regard to its use in children. GBP is
similar in structure to inhibitory neurotransmitter gamma- aminobutyric acid
(GABA) but does not appear to act at the GABA receptor. It is an amino acid that
freely crosses the blood brain barrier. It is not protein bound, it is not
metabolized by the liver, and is excreted solely by the kidneys. Because of a
relative short half-life (5-7 hours) TID dosing is recommended. In children,
dosages of 15-30 mg/kg have been used. GBP is a well-tolerated drug with minimal
side effects. Commonly reported side effects include somnolence, dizziness,
fatigue, and ataxia. Since its general release, other side effects reported
include behavioral changes (aggression, hyperactivity) and weight gain. No
laboratory abnormalities have been reported. Because it is not metabolized by
the liver and because it is not protein bound, it does not interact with other
AEDs. Its absorption can be slowed by antacids, and therefore, it is recommended
that GBP not be taken with these medications. It has been proved effective as
adjunctive treatment of refractory partial seizures and generalized tonic-clonic
seizures. Larger dosages appear to be more effective than smaller ones. In
addition, it may be effective for benign rolandic epilepsy of childhood. Since
the drug is not metabolized by the liver, it may be an effective AED for
patients with porphyria. It is not effective against absence seizures in
children. There appears to be no correlation between serum concentration and
clinical efficacy.
LAMOTRIGINE. LTG is produced by the Burroughs-Wellcome
company and is released as Lamictal®. It is approved for adults as adjunctive
therapy for refractory focal-onset seizures. Despite this limited labeling by
the FDA, LTG appears to have a broad spectrum of activity against multiple
seizure types. In addition, some evidence indicates that it is effective as a
monotherapeutic agent in adults and children for both partial and generalized
epilepsies. Structurally, it is unrelated to other AEDs; its action appears to
be through the voltage-sensitive sodium channels, leading to inhibition of
excitatory neurotransmitter release (glutamate, aspartate). The plasma half-life
is approximately 25 hours, which is halved by enzymatic-inducing AEDs
(carbamazepine, phenytoin) and increased twofold by valproate. Because of this
interaction careful dosing is required. Since the drug has not been labeled for
use in children, no guidelines are provided by the company. Some experts (Goa et
al) have provided some guidelines: for children less than 12 years old taking
enzyme-inducing drugs a starting dose of 2 mg/kg daily for 2 weeks is
recommended followed by 5 mg/kg/day divided BID. A maximum dose of 15 mg/kg not
to exceed 600 mg/day is suggested. If the child is taking valproate alone, a
starting dose of 0.2 mg/kg/day for 2 weeks followed by 0.5 mg/kg/day for 2
weeks, then 1 mg/kg/day with a maximum dose of 5 mg/kg/d not to exceed 200
mg/day is recommended. Common side effects with this agent include dizziness,
ataxia, headache, diplopia, nausea, emesis, somnolence, and rash. Most of the
adverse experiences were mild and transient with relatively few patients
withdrawing from treatment for these reasons in clinical trials. Rashes occur
more frequently in patients receiving concurrent valproate and appear to be
hypersensitivity reactions; rashes are dose-dependent and generally resolve with
reduction or transient withdrawal of the drug. Various behavioral effects
including activation, a sense of “well being” and, more rarely agitation has
been reported. There appears to be no effect of lamotrigine on other AEDs, but
may increase carbamazepine epoxide concentration leading to toxic symptoms.
Despite its approval for adjunctive therapy of partial epilepsy, LTG appears to
be effective for a broad range of seizures types including primary generalized
epilepsy and the Lenox-Gastaut syndrome. A number of monotherapy studies have
demonstrated effectiveness at least equal to that of carbamazepine in the
treatment of focal-onset seizures. In children, effectiveness has been
demonstrated for the generalized epilepsies, including some of the more
refractory types such as myoclonic, akinetic, and tonic seizures. Use in girls
with Rett’s syndrome reported not only improved seizure control but also
improved function and well-being, although how these were assessed was not
clear. The drug is effective at serum concentrations as small as 1-3 µg/ml. The
upper level of clinically tolerable levels has not been established.
VIGABATRIN and TOPIRAMATE. These two new
AEDs have not been approved yet in the United States. Vigabatrin binds to
GABA-transaminase, inhibiting the first step in GABA degradation. It appears to
be effective in the treatment of refractory partial seizures and in the
treatment of West’s syndrome (infantile spasms) in children. Clinical trials in
the the US were delayed initially because of reports of white matter
vacuolization in rats; this change did not appear in higher primates and is of
uncertain significance. In addition, the drug may have significant behavioral
effects. Clinical trials continue. Topiramate is a weak carbonic anhydrase
inhibitor with a number of mechanisms of action. Results from clinical trials in
children have demonstrated that it is a well-tolerated AED effective for both
focal- and generalized-onset seizures. This drug is in the final stages of
approval by the FDA. (Taken from: Clinical Pediatrics; Volume 36, Number 9,
September 1997)
source: http://home.coqui.net/myrna/aed.htm


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