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Alport's Syndrome
• Alport's syndrome is a common cause of hematuria (blood in the urine). It is responsible for about 15% of childhood cases of hematuria.
• Microscopic hematuria: Blood cells in the urine visible only under the microscope, are usually first noticed on routine checks during a routine physical examination.
• Gross hematuria: Red blood cells in the urine are visible with the naked eye when the child has a viral illness, such as a cold or sore throat.
• Slow development of deafness
• Decreases in vision
• High blood pressure
• Swelling of the eyes, especially in the mornings
• Alport's syndrome is caused by a genetic mutation that affects the formation of collagen. Collagen is one of the proteins that helps "hold us together" and is in connective tissue.
• Alport's syndrome is much more common in males than in females.
• Initial diagnosis can be made on the history and physical findings, but confirmation requires a biopsy of the kidney.
• Treatment of Alport's syndrome is supportive, aimed mainly at slowing the progression of the symptoms. The only "cure" is a renal transplant, which is usually undertaken only when a patient's own kidneys have stopped working.
• A low-protein diet is usually recommended to help slow damage to the kidneys.
• High blood pressure is controlled with various medications.
• Unfortunately, there is no therapy available to prevent or slow the development of blindness and deafness.
•
Anal Fissure
• This is a cut (ulcer or laceration) inside the anus. The usual cause is Trauma to the anus because of a strained bowel movement, a hard bowel movement, or high internal sphincter tone (the area that allows stool to pass stays too tense to allow proper passage).
• Severe tearing pain during a bowel movement
• Throbbing pain in anus following a bowel movement
• Blood on toilet paper or around the bowel movement
• Constipation
• Straining
• High internal sphincter tone (explained above)
• Visual inspection by physician
• Tests:
1. Anoscope
2. Flexible sigmoidoscopy
• Sitz baths (baths in a warm tub of water)
• Fiber supplements
• Stool softeners
• Topical anesthetics (Mesalamine suppositories)
• Topical nitroglycerin ointment (at 10% of the strength used for angina) -- relaxes the internal sphincter
• Partial lateral internal sphincterotomy or excision of the fissure
Attention Deficit Disorder
ADD, Attention Deficit Hyperactivity Disorder, ADHD, Attention Deficit, or Hyperactivity Disorder
• Attention deficit disorder is a condition in which an individual exhibits a decreased attention span. He or she can become easily distracted, and have difficulty focusing on tasks. ADD is often associated with hyperactivity -- fidgeting, excessive talking, excessive activity level, impulsive and disruptive behavior. The term ADD and this designation are generally applied to children with this disorder, but may also refer to adults.
• ADD has also become a catch-all classification for a group of symptoms describing undesirable behavior -- sometime eliciting unnecessary medication in children in an attempt to eradicate or modify behavioral problems.
• Lack of attention to details
• Failure to complete tasks
• Easily distracted
• Poor listening skills
• Hyperactivity
• Fidgeting
• Disruptive
• Moves constantly
• Talks excessively
• Interrupts others
• True cause unknown
• It may be associated with dopaminergic, noradrenergic, and serotonergic mechanisms
• Contributing factors:
1. Prematurity
2. Exposure to prenatal toxins
3. Brain injuries
4. Genetic
• Evaluation by psychologist or psychiatrist
• Behavior modification
• Parent counseling
• Psychotherapy
• Medications
1. Dexedrine (amphetamine)
2. Ritalin
3. Cylert
Autism
• Autism is a developmental disorder that causes difficulties in relating to other people. Because we cannot ask children with autism what they are "thinking" when they act certain ways, it is difficult to explain the condition, but many feel that affected children lack the ability to think about the thoughts of others.
• Children with autism have difficulty with imagining the "state of mind" of another person, and often treat people as objects.
• Autism has three main types of symptoms: Impairments in social interactions, impairments in language development, and restrictions in interests and activities.
1. Impaired social interaction:
Lack of play with others: Children will often play "next" to other children without actually interacting with them.
Failure to make eye contact.
2. Impaired language and communication skills:
Repetitive hand and facial gestures that sometimes appear like a "tic," but are much more intricate than a tic
Echolalia, i.e., repeating another person's words and phrases, without regard to their meaning
3. Restricted activities and interests:
Children with autism are very ritual-oriented; disruptions in their daily routines can often provoke behavior problems and outbursts.
Deep interest in one or two subjects, with more communicative patients constantly trying to turn a conversation towards those subjects
Playing with toys by repeatedly stacking them or placing them in lines
• The exact cause of autism is not known at this time.
• Recent studies have identified a possible gene for the disease, but more research is needed.
• Several large studies have shown no connection between autism and vaccinations, or common childhood illnesses.
• The main therapy for autism is behavioral. There are no medications available to treat the disease, and many of the medications that are commonly promoted by some groups (such as the hormone "secretin") can be dangerous.
• Special schooling at an early age can help to reinforce good behaviors and discourage outbursts and behavioral problems.
• The ultimate goal is to move children into regular classrooms and teach them skills that will let them interact with others more effectively.
• Although many children will slowly improve, some will never be able to live and work on their own as adults.
Bell's Palsy
• This is a type of facial paralysis, occurring suddenly on one side of the face. It is thought to be caused by an inflammation of the facial nerve (cranial nerve VII). 60% of afflicted patients get better without treatment, while 90% of those receiving treatment show improvement.
• Since a stroke may have a similar appearance, it is important to make sure that Bell's palsy is truly the cause. Rare tumors can also cause similar symptoms, and these need to be excluded as well.
• Ear pain may precede the attack
• Face feels stiff
• Difficulty closing the eyelids
• Difficulty eating and smiling
• Loss of taste on one side of the tongue
• Hypersensitivity to noises
• Bell's palsy often follows an upper respiratory infection.
• Excessive eye tearing or lack of tearing on one side.
• Unknown
• Exposure to a virus or cold may be the cause in some cases.
• Facial nerve Injury due to head Trauma, tumors, Herpes Zoster, or herpes simplex vesicles in the outer ear canal (Ramsay-Hunt syndrome) can also lead to paralysis of facial nerve.
• Bilateral facial paralysis occurs very rarely, and is often due to neurological disease such as Guillain-Barre syndrome.
• It may be associated with viral infection, such as Epstein-Barr virus, herpes virus, Mumps virus
• Examination:
1. Paralysis of one side of the face
2. One of the sides of the face appears drooped
• Imaging: CT scan or MRI may be done to rule out stroke.
• No treatment is an option, especially if the paralysis is mild.
• Eye patch is helpful if the eye cannot close completely.
• Corticosteroids, e.g., Prednisone
• Corticosteroids work best if they are started sooner than later.
• Lubricating eye drops, if the eyelid remains open.
• Recent studies show that Acyclovir (an anti-viral medication) may be helpful.
• More than 85% of patients get better without treatment.
• 10 % of patients can have mild facial weakness.
• 5% of patients can have permanent severe facial weakness
Biliary Atresia
• Progressive shrinking of the tubes (ducts) which drain bile from the liver to the small intestine
• No known causes, though there is a link to some blood markers
• Occurs 1 per 8,000 to 15,000 births
• Usually seen within the first 6 weeks of life
• Yellow-colored skin, whites of eyes and inside of mouth
• Pale-colored stools
• Enlarged liver, felt as a mass on the right side of the abdomen
• Itching
• Decreased alertness, if prolonged
• Many blood tests are necessary to eliminate other liver disorders.
• Bacterial and viral cultures should be done.
• Eye examination for congenital infections of some viruses
• Ultrasound of the liver and pancreas
• HIV test
• Liver sampling (biopsy) for microscopic evaluation
• X-ray studies for evaluation of congenital problems
• Surgery is helpful but most patients will require a liver transplant, if available.
• Nutritional support is necessary, as food is poorly absorbed.
• Enzymes can be taken orally to help digestion.
• Best results occur when diagnosis is made within the first 2 months of
Dacryostenosis
• Dacryostenosis, or stenosis of the tear duct, is a congenital blockage of the tear duct, occurring in approximately 5% of babies. In these infants, the opening of the tear duct, a passage that drains tears from the eyes into the nose, fails to open before birth. The blockage is inside the nose, at the end of the duct. Because tears cannot escape, they flow back into the eye, where they collect, along with dead cells from the skin of the eyelids, and dirt and dust from the environment. This mixture results in a mucoid discharge in the eyes.
• Because infants do not make large amounts of tears during the first few weeks of life, the condition is often not often recognized immediately after birth.
• Mucous and discharge in the eyes, often collecting in the medial canthus (the edge of the eyelid nearest the nose)
• Tearing of the eyes, with pressure on the junction between the nose and the affected eye
• Lack of redness on the white part of the eye (the sclera) or the inside of the eyelids (the conjunctiva)
• Physical examination is usually sufficient for diagnosis; the lack of any redness of the eyes indicates that the baby does not have an infection.
• Samples of the discharge may be sent to the laboratory if there is a question about the diagnosis.
• In the majority of cases, no treatment is necessary.
• Most infants will outgrow the condition by 9 months of age.
• Although it was once thought to help, massaging the duct after placing a few drops of breast milk into the eye will not hasten the resolution of the blockage.
• In rare cases, in which the duct does not open, a minor operation can open it.
1. An ophthalmologist (eye specialist) will thread a small wire into the duct and open the blockage.
2. This operation is usually postponed until after the first birthday because the risks of anesthesia decline dramatically as the baby grows, and the condition is benign
Branchial Cleft Cysts
Branchial cleft sinus
• This is a defect in the neck present at birth, but sometimes may go unrecognized until adolescence.
• Pits, lumps, or tissue tags on one side of the neck
• Sometimes, fluid drains from a pit on the neck
• It occurs when tissues in the neck area fail to develop properly during the embryonic stage.
• Visual inspection by physician
• Testing is usually not necessary
• It usually requires no treatment.
• Antibiotics for infected branchial cleft
• Total excision for persistent problems
Cellulitis
• Skin infection
• Cellulitis is one type of skin infection
• Cellulitis is a spreading infection of the skin caused by bacteria. The typical causal agents are two: the gram-positive bacteria Staphylococcus aureus ("staph") and Streptococcus ("strep"). Other bacteria may also cause cellulitis, such as the gram-negative Escherichia coli.
• Hot and red spreading rash
• Streaking may appear, going toward the center of the body
• Swollen glands may or may not occur
• Fever, especially in young children and infants
• Cellulitis can be caused by the spread of bacteria from the blood, but it is much more commonly seen after an Injury, such as a cut, that allows bacteria from the environment to gain entry below the skin surface.
• Usually evident on examination
• Culture by saline injection and aspiration (rarely done)
• Antibiotics:
1. The type of antibiotic used depends upon the location and severity of the infection, and the age of the child
2. In young children and infants, cellulitis will often be treated with IV antibiotics, given in a hospital.
3. In older children and teenagers, or with mild infections, oral antibiotics are usually sufficient; however, if the infection spreads despite this therapy, IV medication may be necessary.
4. Cephalexin (Keflex) or dicloxacillin by mouth in typical cases
5. Cefazolin intravenously in severe cases
6. Broad spectrum antibiotics if gram-negative bacteria are suspected (especially diabetics
Cephalohematoma
• This condition refers to a collection of blood under the scalp of the newborn infant.
• The blood "lake" is located between the periosteum (the envelope over a bone) and the bone itself. It does not extend beyond the bone's margin because of the especially tight connection of periosteum-to-bone at the suture line.
• A bulge over part of the infant's skull, usually over the parietal bone on the side of the head; but definitely located over one of the skull bones and not randomly located
• If the blood collection is small, the mass will be soft, mushy.
• If the blood collection is large, the mass will be firm.
• Minor injury during labor and delivery
• At times, cephalopelvic disproportion leads to the injury (that is, the fetal head is large, in comparison to the mother's pelvic outlet)
• An underlying Fracture of a skull bone can sometimes be demonstrated.
• The usual parietal location of the blood collection, on the right or left side of the head, suggests the diagnosis. If the bulging area is soft, then bone can be gently felt underneath (as opposed to feeling a fontanelle underneath).
• Whatever the location on the skull, the bulge should correspond to the outlines of one of the skull bones.
• The bulge does not extend past the outline of one of the skull bones.
• Primiparous mother (mother's first pregnancy)
• Large neonatal birthweight
• At times, forceps delivery
• Watchful waiting is the usual treatment for an uncomplicated case.
• Physicians try not to put a needle into this blood collection because doing so might introduce infection, even with sterile technique.
Choanal Atresia
• A congenital structural disorder of the nose, which consists of membranous or bony tissue in the nasal airway
• It occurs either on one side or on both sides of nose.
• It can be associated with other congenital anomalies of the heart, central nervous system, and genitals.
• One side-type blockage may be asymptomatic until respiratory infection occurs.
• Mouth breathing
• Breathing difficulty when sucking and swallowing
• Bluish lips
• Unknown
• Inability to pass a catheter through each nostril into the throat
• Fiber optic rhinoscopy shows membranous or bony tissue in the nose
• CAT scan
• Maintain the mouth in an open position
• Use orogastric tube or feeding nipple with large holes at the tip
• Operative correction may be delayed until weeks or months later if infant can tolerate mouth breathing.
Cleft Palate
• A birth deformity of the upper lip in which a complete or partial cleft or separation is seen in the upper lip. One-third of cases are thought to be inherited.
• Obstructed breathing
• May cause difficulty with swallowing
• Quality of sounds affected
• The complete cleft palate is obvious.
• The incomplete cleft may require feeling the roof of the mouth for the absence of bone.
• A split uvula (the "punching bag" object in the back of the throat) may suggest an incomplete cleft palate.
• Airway management may be necessary in severe cases.
• The baby may need to be fed with a feeding tube until corrective surgery can be done.
• Speech therapy and dental care may be necessary for normal speech.
Colon Polyp
Polyp, juvenile polyp or retention polyp, intestinal polyp
• A small lump or nubbin of tissue that grows inward, from the bowel wall, into the intestinal tract. A large polyp sometimes blocks the food from passing down the intestinal tract.
• The commonest symptom is bloody stool (bowel movement). This is bright red in appearance and painless in most cases.
• The usual age for this symptom to appear is between the ages of 3-5 years.
• Unknown
• It may be caused by defective fetal tissue, when that tissue is forming the intestinal tract.
• History of bright red blood in the stool
• Examination of the colon (large intestine) with a rectal instrument, called a sigmoidoscope, is usually successful in diagnosing this.
• Inherited condition (genetic)
• Removal of the polyp by passing a small grasping instrument through the sigmoidoscope
Congenital Muscular Torticollis
"Wry neck"
• Torticollis is an abnormal positioning of the head.
• Head positioned in the characteristic fashion:
1. The head is tilted toward the affected side.
2. The chin is turned away from the affected side.
3. Because of this, the child appears to be looking up and to the side; the child looks away from the side that is affected by the problem.
4. Parents usually notice that a baby will not look in one particular.
• Neck mass on the affected side:
1. The mass is usually first noticed soon after birth, but sometimes may not be found until several weeks after birth, when the infant's neck begins to elongate.
2. The mass is firm and not tender, and increases in size for several weeks, then begins to slowly disappear over several months.
• The cause of congenital muscular torticollis is not known. However, there are several prominent theories:
1. Injury to the neck during the delivery process
2. Intrauterine pressure on the neck due to positioning in the uterus (womb)
3. Abnormalities of blood vessels and blood flow in the fetus
4. These abnormalities result in the formation of a small scar inside the muscles of the neck. It is this scar that is felt as the neck "mass." The scar tissue prevents the muscle involved from working normally.
• Diagnosis is made on the basis of physical examination and history; no tests are needed.
• However, because some children with this condition will also have other musculoskeletal problems, x-rays of other parts of the body may be required.
• If the neck mass fails to disappear, an ultrasound may help confirm that other problems are not present.
• Slightly more common in males than in females
• About 10% of babies with congenital muscular torticollis also have a family member with torticollis.
• The mainstay of treatment is stimulating the infant to turn his/her head toward the affected side. This will stretch out the scar tissue in the involved muscle.
1. Usually, this is accomplished by placing the baby in a crib with all the toys and bright objects on the affected side. This forces the infant to turn the head to look at the interesting things in his/her environment.
2. Some range-of-motion and stretching exercises are sometimes also used.
3. If done consistently, these interventions will work in nearly all affected babies.
• The small number of children in whom the condition persists past the first birthday, or in whom the loss of range-of-motion is profound, require surgery to remove the scar tissue and return full function to the affected muscle
Constitutional delayed puberty
more about Constitutional delayed puberty
Constitutional growth delay, physiologic delay of puberty
• The change in the physical appearance of the child is delayed, as he or she becomes a teenager and then an adult. The development of breasts, muscles, and the development of body hair and sex organs may be delayed.
• The normal body changes occur several years later than normal.
• Expressed as a lack of common signs of puberty
• More than half of all cases have other family members with the same tendency.
• This can just be a variation of normal. Not every person matures in physical appearance at the identical age as his or her classmates.
• Persistence of low level gonadotropin of childhood
• Genetic factors, or the condition may be a family tendency
• Low level of gonadotropin
• Measurement of the amount of growth hormone or other hormones, in the blood.
• Evaluation of chromosomes of the individual
• Physical examination of the individual, including examining the genitals and breasts
• History of drug use (steroids)
• 90% of cases are just a variation of normal.
• Most patients with this condition do not require medication.
• Sometimes, hormone therapy is used.
Cryptorchidism
"Hidden testicles"
• This condition occurs when the testicles are not present in the scrotum at birth. They may be present in the abdominal area (and undetectable by physical exam), or anywhere between the abdomen and the scrotum.
• The higher temperatures in the abdomen can permanently damage the testicles and, therefore, it is ideal to correct this condition early.
• Normal testicles descend at 7 months of gestation.
• Cryptorchidism occurs in 3.5% of term infants, 17% of premature infants, and in 100% of infants weighing less than 900 grams.
• None, other than apparently absent testicle(s)
• Examination for the presence of both testicles in the scrotum
• If absent, ultrasound or magnetic resonance imaging (MRI) should be done to locate one or both testicles.
• Rarely, a surgical inspection or sample is required.
• If partially descended, hormonal treatment with human chorionic gonadotropin (HCG) may be successful.
• If treatment is unsuccessful by 6 months of age, surgery may be necessary to either re-locate or remove the hidden testicle(s).
Developmental Hip Dysplasia
Developmental dysplasia of the hip (DDH), Congenital hip dislocation, Subluxation of the hip
• Any hip abnormality, originating during fetal development
• Asymmetrical appearance, or asymmetrical tone of the lower extremities.
• Limited hip abduction occurs on the affected side. Both right and left lower extremities might be affected in DDH. Therefore, both sides might show limitation of hip abduction.
• Heredity
• Unknown
• Physical examination (Barlow test, Ortolani test) showing dislocatable or dislocated hip
• Ultrasound examination of the hip joints
• X-rays of the hip joints (AP and frog views)
• Female sex
• Breech presentation
• Primiparous mother (i.e., first pregnancy)
• Newborns are frequently treated with triple diapers to hold the hips in a physiologic position.
• Older infants and children might be treated with a brace or cast, to hold the hips in a physiologic position.
• Surgery in selected cases
Disseminated Intravascular Coagulopathy
more about Disseminated Intravascular Coagulopathy
DIC, Consumption coagulopathy
• Activation of the clotting cascade within the blood vessels causes the ordinarily liquid blood to clot. The utilization of a large percentage of the available clotting factors leads to generalized bleeding.
• Prolonged bleeding from any site
• Sudden appearance of multiple ecchymoses (black-and-blue marks)
• Petechial rash
• Any process that activates the clotting cascade
• Microscopic examination of a blood smear shows fragmented RBC's and decreased numbers of platelets.
• Abnormal blood tests:
• Increased fibrin split products
• Prothrombin time (PT) and partial thromboplastin time (PTT) are prolonged.
• Serious infection (viral, bacterial, rickettsial)
• Serious Trauma
• Shock
• Cancer
• Incompatible blood transfusion
• Severe liver disease
• Various treatment plans in various situations:
• Fresh frozen plasma, to replace several clotting factors that have been consumed
• Cryoprecipitate
• Platelet transfusions
• Heparin
Epididymitis
• The epididymides are coiled tubes that bring sperm from the testicles to the urethra (urethra is the passage way that runs the length of the penis). These tubes in the epididymes are located just above the testicles. When an infection occurs in the epididymides, it is called epididymitis.
• It is rare before puberty.
• Pain in the scrotum
• Scrotal swelling
• Penile discharge
• Pain at tip of penis
• Fever
• Pain when urinating
• Too frequent urination
• Sexually Transmitted Disease (STD), such as the following, is often the cause in adults under the age of 35:
1. Gonorrhea
2. Chlamydia
• Non-sexually transmitted:
1. Gram-negative bacteria/urinary tract infections
2. Tuberculosis.
3. Certain drugs such as amiodarone
4. Congenital (present at birth) abnormalities
• Examination
1. Tenderness above the testicles
2. The pain is reduced with lifting the scrotum.
• A microscopic urine exam shows bacteria and elevated white blood cell count in the urine.
• Gram stain and culture of penile discharge
• Ultrasound of scrotum
• Immediate antibiotic therapy
• Start with ampicillin and then change based on the culture and sensitivity
• Sexually-transmitted forms: A one-time ceftriaxone injection and doxycycline by mouth for 10 days.
• Non-sexually transmitted: Bactrim, Cipro, or Floxin for 21-28 days
• Sitz baths (warm tub baths)
• Scrotal support
• Treatment of sexual partners if it is a sexually transmitted disease
Testicular Torsion
• Testicular torsion occurs when a testicle gets twisted around, cutting off its blood supply. The patient with this condition usually has severe pain in the testicle.
• This is an emergency situation and requires immediate attention at the hospital. Risks may include injury to the testicle and dead tissue in the testicle. Infection of the damaged tissue can also occur. In children, torsion usually occurs in boys under 6 years of age.
• Usually severe testicular pain that begins suddenly.
• The testicle is swollen and very tender.
• The pain often travels along the inside of the thigh in spasms.
• Urine tests are often normal, without any evidence of an infection.
• This problem usually occurs in young, healthy boys up to 20 years of age.
• Examination by the doctor may show that the affected testicle is higher up than normal.
• The disease is caused when the testicle gets twisted, cutting off the blood supply to the affected testicle.
• The diagnosis is usually suggested by the symptoms.
• Ultrasound of the testicles may help in making the diagnosis.
• This is an emergency and the patient needs immediate medical attention.
• Usually, a surgeon or urologist is called in to see the patient and decide the best treatment (surgical exploration or orchiectomy)
• Delaying getting medical care can cause serious complications.
• Do not delay. Go to the emergency room immediately.
• Examination of the opposite testicle is also necessary.
Epistaxis
Nosebleeds
• Bleeding from the nose
• It is common in childhood.
• Bleeding from one nostril or occasionally from both
• Child may vomit blood or blood may appear in stool when child swallows blood from a nosebleed.
• Picking nose
• Foreign body in nose
• Vigorous nose blowing
• Broken nose
• Barotrauma
• It can be associated with Upper Respiratory Infection, Sinusitis, Allergic Rhinitis, adenoid hypertrophy, or polyps.
• Chemical irritants
• Congenital vascular abnormalities such as telangiectasias, varicosities
• Thrombocytopenia
• Deficiency of clotting factors
• Hypertension
• Renal failure
• Most stop without treatment.
• Keep the head tilted forward.
• Compress the soft portion of the nose.
• Apply ice on the bridge of the nose
• Local application of Neosynephrine
• Nasal packing if bleeding persists
• Cautery with silver nitrate
• Blood transfusion when severe bleeding
Gingival cyst
• This is a temporary accumulation of epithelial cells on the gums and the roof of the mouth.
• It occurs in newborns.
• Whitish-yellow solid bumps on the gums and the roof of the mouth, which sometimes resemble emerging teeth.
• It requires no treatment.
• It disappears within a few weeks.
Erb's Palsy
Erb-Duchenne palsy or paralysis
• Erb's palsy is a weakness of the arm that occurs in newborns. It is caused when the nerves that control the arm are injured during the birth process.
• Infants with Erb's palsy hold the affected arm at the side, with their forearm turned inward and their wrist bent. When a baby with Erb's Palsy is stimulated, the affected arm moves less than the unaffected arm.
• Erb's palsy is commonly associated with shoulder dystocia. Shoulder dystocia occurs when an infant's shoulder gets caught and stretched behind the pelvic bone during delivery.
• The condition can occur, albeit rarely, without being caused by shoulder dystocia.
• Diagnosis is made clinically.
• If there is a question regarding the reason for the weakness, X-rays can rule out a fracture.
• Larger babies are at a higher risk for deliveries that involve damage to their shoulder, and thus at higher risk for Erb's palsy. For the same reason, mothers who have a smaller pelvic opening will also have a greater risk for delivering an infant with Erb's palsy.
• Other risk factors include maternal diabetes, prolonged labor, an infant that presents in the breech position or induction of labor.
• Although an affected infant's arm is often placed in a sling for several weeks to prevent further stretching or rupturing of the affected nerves, specialized therapy isn't the usual course of action.
• After a period of healing, stretching and range of motion exercises are prequently performed to help the child recover flexibility.
• Surgical intervention followed by physical therapy may be necessary when a baby demonstrates no functional recovery by four months of age
Fetal Alcohol Syndrome
• The manifestations of specific growth, mental, and physical birth defects associated with the mother's high level of alcohol use during pregnancy are as follows:
1. Small birth weight, small head circumference
2. Epicanthal folds; small, widely-spaced eyes; flat mid-face
3. Short, upturned nose; smooth, wide philtrum; thin upper lip (the philtrum is the space between the bottom of the nose, and the top of the upper lip)
4. Underdeveloped jaw
5. Irritability, difficulty eating or sleeping, hypersensitivity to any form of stimulation
• Neurological manifestations of FAS:
1. Attention deficits, memory deficits, hyperactive
2. Difficulty with abstract concepts (math, time, money)
3. Poor problem solving skills, difficulty learning from consequences
4. Poor judgment, immature behavior, poor impulse control
• There is no cure for Fetal Alcohol Syndrome. Once the damage is done, it cannot be undone. However, FAS is the only cause of birth defects that can be completely prevented
Foreign Body Aspiration
Inhalation of a foreign object; obstructed airway
• A condition in which a foreign object is inhaled and lodged into the lower respiratory tract, via the mouth or nose, causing respiratory problems.
• It usually occurs in children who are between 6 months and 3 years of age.
• It occurs in boys twice as often as girls.
• Aspiration may cause only minimal initial symptoms
• Coughing
• Wheezing
• Harsh sound on inhalation
• Shortness of breath
• Coughing up blood
• A peanut is the most common aspirated foreign object.
• Hot dogs, balloons, small toys, buttons, beads, coins, marbles, pins, screws, rocks, and anything else small enough for children to put in their mouth or nose.
• History:
1. Choking (object in airway) episode in only 50 - 80 % of cases
2. Symptoms such as coughing, wheezing. But initial symptoms can be minimal until a lung infection presents.
3. Pneumonia that does not respond to antibiotics treatment
• Physical exam:
1. Locally diminished or absent breath sounds in 50 % of cases
2. Increased heart and respiration rate (i.e., number of breaths per minute)
• Images of the lungs:
1. Chest X-ray, inspiratory and expiratory X-ray: These may show air trapping, mediastinal shift, atelectasis, or lung infection.
2. Fluoroscopy
• Rigid bronchoscopy for definitive diagnosis
• Administering oxygen
• Rigid bronchoscopy to remove the object
• Antibiotics
• Chest physiotherapy after endoscopy
Foreign Body in the Vagina
• Young females may insert foreign objects into the vagina.
• It usually occurs in girls less than 4 years of age.
• Vaginal bleeding
• A foul-smelling vaginal discharge
• Pain in the groin , swelling
• Painful urination
• Toilet paper is most common foreign body inserted in the vagina.
• Crayons
• Beads
• Pins
• Buttons
• Based on history, physical examination
• A plain X-ray or ultrasound of the pelvis
• Removal of the foreign body (do not attempt by yourself)
• Control the bleeding
Hemolytic-Uremic Syndrome
HUS
• Hemolytic-uremic syndrome is a disease in which the number of platelets suddenly decreases, and the body's red blood cells are destroyed. Kidney failure occurs simultaneously. This is a rare syndrome that can occur after bacterial infection or develop from other diseases. Some research suggests a genetic basis to the disease, but the exact cause is unknown. It occurs most often in infants, small children, and pregnant women, but can occur in older children, adults, and women who aren't pregnant.
• Irritability, restlessness
• Fatigue
• Shortness of breath
• Pallor
• Rectal bleeding
• Leg swelling
• Decreased urine amount
• Seizure
• Diarrheas:
1. E. coli 0157:H7
2. Shigella
3. Salmonella
4. Viral
• Familial (genetic)
• Pregnancy (especially immediately after pregnancy)
• Extremely severe (malignant) high blood pressure
• After a kidney transplant
• Manual blood count shows hemolytic anemia in visual inspection of the red blood cells
• Decreased platelets
• LDH level is high
• Coombs test is negative
• Elevated fibrin level
• Elevated blood urea nitrogen (BUN) level
• Children usually recover, so treatment is supportive. Dialysis at intervals may be required until healing is complete.
• Adults undergo large volume plasmapheresis (plasma fraction of the blood is exchanged).
• Maintain Calories
• For kidney problems:
1. Restrict fluid
2. Diuretics
3. Correction of electrolytes disturbance
4. Dialysis
5. Antihypertensive agents such as hydralazine, captopril, or nifedipine
• For bleeding problems: Transfusion for symptomatic bleeding
• Plasmapheresis (plasma fraction of the blood is exchanged)
Systemic lupus erythematosus
• This is an autoimmune disease (the body's immune system mistakenly attacks itself). Almost every organ in the body is susceptible to this disease.
• It affects the joints (causes arthritis) in 90% of those infected with the disease.
• Joint pain, stiffness or swelling
• Fever
• Weight loss
• Fatigue
• "Butterfly rash" -- red rash on the cheeks
• Skin lesions
• Sensitive to light
• Eye inflammation/redness
• Vision problems
• Shortness of breath
• Cough (severe cases with blood)
• Palpitations
• Abdominal pain
• Psychosis
• Seizures
• Depression
• Kidney failure (leg swelling)
• Anti-nuclear antibody is usually positive (though not specific)
• Anti-double stranded DNA antibodies and anti-Sm antibodies are relatively specific for lupus (but not as sensitive)
• Antiphospholipid antibodies may occur. These increase the risk of blood clots and miscarriages.
• 85% female predominance; this predominance is 3:1 in prepubertal patients.
• Generally occurs from 15 to 50 years of age
• Most commonly found in African-Americans, Asians, Native Americans and Hispanics
• Familial (genetic) factors -- common genetic markers for SLE are the genes for HLA-B8, DR2 and DR3. There may be other markers for this disease (complement deficiencies and polymorphisms of the Fc gene).
• No specific therapy
• Mild -- supportive only
• Anti-inflammatories, e.g., ibuprofen
• Hydroxychloroquine (person should be monitored for retinal damage by an ophthalmologist)
• Corticosteroids (e.g., prednisone) for more severe cases.
• Cyclophosphamide, chlorambucil or azathioprine may be considered for cases resistant to prednisone.
• If antiphospholipid antibodies are present and clotting occurs in arterial or venous systems, then Coumadin is prescribed.
• Dialysis and renal transplantation
Sepsis
Septicemia; "Blood poisoning"
• The most common occurrences of pediatric sepsis are (1) in the neonatal period (first 30 days of life); (2) in post-splenectomy patients; and (3) in meningitis.
• Ordinarily, common usage refers to bacterial infection of the bloodstream, causing symptoms, and would exclude viral infection. A well-known fungal infection of the bloodstream, caused by yeast (candida) is known as candida sepsis, so the term, "sepsis," is not strictly limited to bacterial causes.
• Sepsis is a serious condition.
• Infection may be acquired in-utero, or during labor. Alternatively, infection may be acquired post-natally.
• Post-natal symptoms in neonates:
1. Poor suck, poor feeding, weakness, irritability
2. Poor weight gain in the nursery, numerous other symptoms
3. Temperature instability, including sub-normal temperature
4. Pallor, gray color, jaundice, abnormal vital signs, respiratory distress, enlargement of liver and/or spleen, Diarrhea
5. Alternatively, a septic neonate might have no symptoms or signs.
• First 30 days of life:
1. Group B Streptococcus
2. E. coli
3. L. monocytogenes
• Older children:
1. S. Pneumonia
2. Group A Streptococcus
3. Staphylococcus
• Because infection can occur in-utero or during labor, it is important to note the presence of the ante-natal risk factors above.
• Diagnosis is determined by the collection of symptoms and signs, and supported by laboratory data such as the WBC (white blood cell) count and differential, and any positive blood cultures.
• Ante-natal (prenatal, prior to birth) risk factors:
1. Premature rupture of membranes
2. Group B Streptococcal infection in the mother
3. Maternal fever
4. Cloudy, foul-smelling amniotic fluid
• Post-natal (after birth) risk factors:
1. Presence of catheters, tubes, drains
2. Antecedent deep infections (Pneumonia, meningitis, Urinary Tract Infection)
• Ordinarily, antibiotics or antifungals intravenously
• Supportive measures
Hemophilia
• Factor VIII deficiency (Factor 8 deficiency), or Hemophilia A, is the commonly called Hemophila.
• Christmas disease (hemophilia B) (Factor 9 deficiency), has the same symptoms but is rarer. Only about 15% of all Hemophila cases are Hemophilia B.
• One of the ways that blood clots is through a cascade of factors. Hemophilia is a bleeding disorder caused by a deficiency of blood clotting factors.
• It is genetically inherited and almost exclusively affects males.
• It can be divided into hemophilia A and hemophilia B (also called Christmas Disease); hemophilia A is much more common (accounting for 80% of all cases).
• Bleeding in the joints: knees, ankles, or elbows.
• Bleeding into the muscles
• Bleeding from the rectum or vomiting blood
• Severe hemophilia: less than 1% Factor VIII or Factor IX
- Spontaneous bleeding or bleeding with very minor Trauma
• Moderate hemophilia: between 1% and 5% Factor VIII or Factor IX
- Bleeding with moderate injury
• Mild hemophilia: more than 1% Factor VIII or Factor IX
- Bleeding only with significant Trauma
• Lack of production of clotting factors
1. Low levels of Factor VIII -- hemophilia A
2. Low levels of Factor IX -- hemophilia B
3. Low levels of Factor XI (Factor eleven) is sometimes called Hemophilia C.
• Factor VIII and Factor IX assays: Decreased Factor VIII (hemophilia A) or Factor IX (hemophilia B)
• There are mild, moderate, and severe forms of hemophilia A, depending the depletion levels of Factor VIII.
• Prolonged activated partial thromboplastin time (APTT)
• HIV testing -- a high number of persons with hemophilia developed HIV due to transfusions of missing factors which are blood products (occurred in the time before proper screening)
• Replacement treatment
1. Factor VIII concentrate transfusions (now heat-treated to reduce HIV transmission). The goal level of Factor VIII depends on the severity of the bleeding problems being treated
2. Factor IX concentrate transfusions for hemophilia B
• Desmopressin acetate (DDAVP) -- for mild or moderate hemophilia A
• Aminocaproic acid, Tranexamic acid for oral bleeding
• Avoid aspirin and anti-inflammatory medications (e.g., ibuprofen)
Henoch-Schoenlein Purpura
Schoenlein-Henoch purpura, Anaphylactoid purpura, Vascular purpura, Allergic purpura
• This is an inflammatory response within the blood vessels and/or capillaries.
• It is characterized by purple spots on the skin, joint pain, gastrointestinal symptoms, and kidney disorders.
• It usually occurs in children ages 2-8.
• It is more common in boys than girls.
• Primarily, redness and swelling of the skin caused by congestion of the capillaries.
• Purple spots (wheals) -- usually on the lower extremities and buttocks, but may involve elbows, trunk, and face
• Lack of energy
• Low-grade fever
• Joint pains
• Nausea/vomiting
• Abdominal pain
• Diarrhea
• Bloody stools
• Painful Menstruation
• The exact cause is unknown, but it is related to an abnormal response of the immune system.
• It can be associated with allergy or drug sensitivity.
• Based on physical examination
• Blood tests show increased white blood cells, increased eosinophils, and elevated erythrocyte sedimentation rate (ESR).
• Urine tests show microscopic blood in the urine.
• Skin biopsy shows vasculitis.
• Symptomatic treatment.
• Nonsteroidal anti-inflammatory drugs
• Corticosteroid therapy for central nervous system manifestations, nephritic syndrome, or acute phase complications, such as intestinal hemorrhage, obstruction, or perforation.
• Azathioprine or cyclophosphamide for severe nephritis
Hydrocele
• A hydrocele is a collection of fluid surrounding one of the testicles. The majority of hydroceles are congenital (present at birth).
• There are two forms of hydrocele: communicating, and noncommunicating.
1. In a communicating hydrocele, there is an open connection between the scrotum and the abdominal cavity; this kind of hydrocele increases the risk of developing a hernia.
2. In a noncommunicating hydrocele, no open connection exists, and there is no increased risk of development of a hernia.
• The symptoms and therapy are different for the two forms of the disease.
• Enlargement of one side of the scrotum (both communicating and noncommunicating forms)
• Variation in size of the enlargement at different times of the day (communicating)
• On physical examination, the physician can shine a flashlight through the mass in the scrotum to make sure it is fluid-filled and not solid.
Hydrops Fetalis
RH Incompatibility, Rh-induced hemolytic disease of the newborn
• This is severe form of hemolytic disease of newborn.
• This condition occurs when there is a difference in the mother's and fetus's Rh blood type.
• First-born infants are unaffected (unless the mother has had previous interrupted pregnancies that could have sensitized her system), as it takes time for the mother to develop antibodies against the fetal blood.
• Anemia
• Heart failure
• Edema
• Respiratory distress
• Enlarged liver
• Bruising or purplish bruise-like skin lesions
• Rh-negative mother makes antibodies against the red blood cells of fetus who is Rh-positive.
• These anti-Rh positive antibodies move through the placenta to the fetus and destroy the fetus's circulating red blood cells.
• Destroyed red blood cells release free hemoglobin into the infant's circulation. Hemoglobin is converted into bilirubin.
• High bilirubin level
• Positive direct Coombs test
• Evidence of hemolysis in the infant's blood
• Elevated cord blood bilirubin
• Increase fluids
• Phototherapy
• Intrauterine fetal transfusion, early induction of labor for hydrops fetalis
• Transfusion of packed red blood cells
• Exchange transfusion
• An injection of immunoglobulins, called RhoGAM, to Rh-negative mothers at a mid-term and a second injection within a few days of delivery usually prevents the development of antibodies against Rh-positive blood.
Pyloric Stenosis
Normal
Abnormal
• Pyloric Stenosis is caused by an enlargement of the muscles that close the tube (sphincter) that empties the stomach.
• Babies with Pyloric Stenosis appear normal at birth, but develop symptoms 6 to 8 weeks afterwards.
• Pyloric Stenosis is more common in first-born male infants, but it can occur in both males and females. It is also more common in babies who receive oral erythromycin, an antibiotic, during the first few months of life. The exact cause of the muscular enlargement is not known.
• "Projectile" vomiting
• Forceful vomiting of large volumes, unlike "spitting up," which dribbles out from the mouth in small amounts
• Vomiting occurs a short time after feeding.
• Babies appear very hungry and start to feed very quickly, but then push the bottle away
• With prolonged vomiting, Dehydration can occur, leading to decreased urination, lack of tears, and a dry mouth.
• Examination of the abdomen
• If diagnosis is uncertain, Ultrasound or X-Ray studies are performed.
• Blood studies sometimes show changes in electrolyte (mineral) levels.
• Surgery is required to enlarge the opening between the stomach and the intestines.
• Most infants go home from the hospital in 1 or 2 days.
• If left untreated, frequent vomiting can lead to Dehydration and electrolyte changes in the blood.
• Most cases are thought to be congenital, so prevention is not possible. However, because of the association with erythromycin use, most pediatricians will use this medicine in infants under 2 months of age only when absolutely necessary.
Hirschsprung's Disease (Megacolon)
People with Hirschsprung's disease lack the nerve cells that enable intestinal muscles to move stool through the large intestine (colon). Stool becomes trapped in the colon, filling the colon and causing it to expand to larger than normal. Hirschsprung's disease is also called megacolon. It is a congenital disease, which means a person is born with it. The disease may also be hereditary, which means a parent can pass it to a child. Hirschsprung's disease affects mainly infants and children.
Although symptoms usually begin within a few days after birth, some people don't develop them until childhood or even adulthood. In infants, the primary symptom is not passing meconium, an infant's first bowel movement, within the first 24 to 48 hours of life. Other symptoms include constipation, abdominal swelling, and vomiting. Symptoms in older children include passing small watery stools, diarrhea, and a lack of appetite.
Physicians diagnose Hirschsprung's disease through rectal manometry, a lower gastrointestinal (GI) series, and rectal biopsy. Rectal manometry involves recording pressure changes within the colon and rectum. In a lower GI series, x-rays are used to measure the width of the colon and rectum. Rectal biopsy involves removing a piece of rectal tissue to learn whether the nerve cells that control intestinal muscle contractions are present.
Colostomy is the most effective treatment for Hirschsprung's disease. In a colostomy, the surgeon removes the affected part of the colon. The top half of the remaining colon is then connected to a surgically created hole, called a stoma, on the abdomen. Stool can leave the body through this hole while the lower part of the colon heals. Later, the surgeon will reconnect the colon inside the body and close the stoma. The patient will then be able to have normal bowel movements.
Intussusception
• Intussusception is a condition in which one part of the intestines slides into the next lower part.
• Intussusception is one of the most common causes of Intestinal Obstruction in infants. It can occur at any age, but usually affects infants 1 to 2 years old. It is rare in those older than 2. It happens much more often in boys than girls. In this condition, a part of the intestines slides into the next lower part. That is, there is a "telescoping" effect. This can lead to abdominal pain, obstruction, bleeding, and potentially may compromise the blood supply to the bowel. Treatment needs to be started immediately. Sometimes a Barium Enema will not only diagnose the problem, but also treat it. At other times, surgery may be necessary.
• Intussusception usually begins with a sudden onset of stomach pain that causes the child to cry and pull his legs up to his chest. Initially, the pain may come and go. However, as time passes, the pain becomes more severe and lasts longer.
• Vomiting
• Stool that looks like "currant jelly." (This is the classic description of the stool passed by individuals with Intussusception, in which blood and mucous mix to produce this distinctive appearance.)
• Depending on the severity of the problem, there may be fever, Dehydration, and/or shock.
• Weakness and restlessness
• Stomach Bloating, distention
• On exam, the doctor may notice high-pitched bowel sounds. Also, a fullness or mass may be present in the upper right part of the abdomen
• The physical exam may reveal an abdominal mass. The abdomen is usually tender to the touch.
• Vital signs may show an elevated heart rate, fever, or Low Blood Pressure.
• Abdominal X-Rays may show an obstruction.
• One of the best tests is a Barium Enema.
• An abdominal CT scan can also help in making the diagnosis.
• If the diagnosis is still not certain, a Laparoscopy can be done. In this procedure, a camera is inserted into the abdominal cavity so that the surgeon can look at the intestines and identify what is causing the problem.
Necrotizing Enterocolitis
Topic Overview
What is necrotizing enterocolitis?
Necrotizing enterocolitis is infection and inflammation of the intestines. The disease usually occurs in premature newborns. Many newborns who develop necrotizing enterocolitis survive and go on to live healthy lives. However, if the infection becomes severe, it can cause serious damage to or holes in the intestinal tissue, which can be life-threatening.
Necrotizing enterocolitis most commonly develops 10 to 16 days after birth, usually while a premature newborn is still in the hospital. Occasionally, necrotizing enterocolitis develops up to 3 months after birth.
See an illustration of necrotizing enterocolitis .
What causes necrotizing enterocolitis?
The cause of necrotizing enterocolitis is not clear. It is believed to occur when the immune and digestive systems do not develop properly. This can happen when a baby is born prematurely or when there are complications during pregnancy or delivery.
Experts do not know if feeding a newborn formula can lead to necrotizing enterocolitis, but they do know that the disease is much less common in babies who are fed breast milk.
What are the symptoms?
Often, a newborn baby is feeding well, healthy, and growing before he or she develops any symptoms of necrotizing enterocolitis. A baby's symptoms depend on how severe the condition is. If your baby has necrotizing enterocolitis, he or she may:
• Have a swollen, tender, red, or shiny belly.
• Not want to eat or be throwing up.
• Be constipated.
• Have dark, black, or bloody stools.
• Have low or unstable body temperature.
• Not be very active or have little energy.
How is necrotizing enterocolitis diagnosed?
Necrotizing enterocolitis is diagnosed from a newborn's symptoms, medical history, and test results. Tests may include an abdominal X-ray to provide a picture of your newborn's intestines; a test to check for blood in the stool (fecal occult blood test); and other tests to check for bacteria in the stool, blood, urine, or spinal fluid.
How is it treated?
If your newborn has necrotizing enterocolitis, he or she will need to be treated in a hospital, often in a neonatal intensive care unit (NICU). In newborns who have mild to moderate necrotizing enterocolitis, treatment consists of intravenous (IV) feeding, antibiotics, and removing extra fluids and gas from the intestine. This treatment usually lasts between 3 and 10 days.
If your newborn does not improve with treatment, or if he or she develops a hole in the intestines, surgery to remove damaged parts of the intestines may be necessary. About 25% to 50% of newborns with necrotizing enterocolitis need surgery.1 Many newborns who have surgery for necrotizing enterocolitis survive and go on to live healthy lives.
What is meconium ileus?
Meconium is the first stool (bowel movement) that a newborn has. This stool is very thick and sticky. Meconium ileus is a bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum. Most infants with meconium ileus have a disease called cystic fibrosis.
Volvulus
• A twisting of the intestines around themselves or around other bands of tissue
• Can cause complete obstruction or incomplete obstruction
• Sometimes associated with abnormal rotation of the intestines
• In complete obstructions, severe pain, vomiting, fever, bloody stools
• In incomplete obstructions, feeding intolerance, nausea, irritability, frequent episodes of abdominal pain
• Volvulus most common in newborn and infants several months of age
• X-rays can show distended stomach and intestines
• Abdominal tenderness, bloody stools, fetal position posturing, fever, and rapid heartbeat
• Hospitalization for surgery to relieve the twisting and obstruction
• Oftentimes, parts of the intestines must be removed.
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| hanwin
Forum Senior
Topics: 6
Posts: 157
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Very useful. Many thanks.
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