A married couple brings their newly adopted 5-month-old for his first well child visit. They ask when the child can sit in a safety seat in the car facing forward. You would advise that the child should face rearward until he:
Correct Answer A:
A child can start riding facing forward when he or she is at least 10 kg (22 lb.). To prevent the car seat from moving forward and causing injury in a collision, it is important to use the tether strap exactly as the manufacturer recommends. The shoulder straps should be at or above the child's shoulders. Straps should be snug, with only one finger width between the strap and the child's chest. Using aftermarket car seat products should be avoided. They can become projectiles or may have hard or sharp surfaces that can hurt the child in a collision.
Infants who weigh less than 10 kg at one year of age should continue to ride in the rearfacing position.
The mother of an 8-year-old female is concerned about purple “warts” on her daughter’s hands. The mother explains that the lesions started a few months ago on the right hand along the top of most of the knuckles and interphalangeal joints, and she has recently noticed them on the left hand. The child has no other complaints and the mother denies any unusual behaviors. A physical examination is unremarkable except for the slightly violaceous, flat-topped lesions the mother described.
What is the most likely cause for this patient’s finger lesions?
One of the most characteristic findings in dermatomyositis is Gottron’s papules, which are flat-topped, sometimes violaceous papules that often occur on most, if not all, of the knuckles and interphalangeal joints.
A 6-year-old boy was brought to the hospital because of a two-month history of lassitude and weakness. At first, he had difficulty climbing stairs. For the last two months, he had to roll over on his side to get off the bed and use his hands on thighs to stand from the sitting position. For the last two weeks he asked for help to comb his hair. There are no other complaints and the family history was unremarkable. Routine vaccination was up to date. On examination, the child looked well, not pale, jaundiced or cyanosed and he was afebrile. His upper eye lids had purplish colored macules and the dorsal surface of his interphalangeal joints had flat-topped pinkish papules. His cheeks, forehead and the dorsal surfaces of the forearms were erythematous. Positive Gower sign was confirmed. Slight tenderness was also noted over the hamstring and quadriceps muscles. Tendon reflexes were normal and sensations were intact. Electromyography showed increased insertional activity, fibrillation potentials, sharp waves and decreased duration and amplitude of action potential. Motor and sensory conduction velocities were however, normal. Creatine kinase was 3000 U/L.
Given this patient's presentation, what would be the most likely diagnosis?
Correct Answer D:
Dermatomyositis, an inflammatory disorder of muscle, is a rare condition affecting 3 per 1,000,000 children with peak onset between 4 and 10 years. It is relatively more common in girls.
Dermatomyositis presents with skin rash or insidious onset weakness or both. The typical rash is heliotrope rash, a blue-violet discoloration of the upper eyelids. Another typical type of rash is the flat topped pinkish classic Gottron’s papules on the back of the interphalangeal joints. The weakness usually affects proximal muscles such as neck flexors, shoulder girdle and hip flexors. Patients may thus, have difficulty in climbing stairs, standing from the sitting position, combing hair or getting out of bed. These children may be observed to use hands on thighs to stand from the sitting position (Gower sign). Tenderness of muscle is one sign of muscle inflammation in patients with dermatomyositis. Lab findings include high serum creatine kinase and myopathy EMG changes (increased insertional activity, fibrillation potentials, sharp waves and decreased duration and amplitude of action potential). Motor and sensory conduction velocities are typically normal in patients with dermatomyositis. Our patient has most of these findings.
→ In acute intermittent porphyria (AIP), in addition to muscle weakness, there is also sensory involvement that might be described by patients as "muscle pain." The muscle weakness of AIP is neuropathic in nature. Thus, EMG is expected to show either low motor and sensory conduction velocities or low amplitude of action over muscle. Increased insertional activity, fibrillation potentials, and sharp waves typical of myopathy do not develop in AIP. Creatine kinase is expected to be normal in this condition.
→ The main findings against the diagnosis of Duchenne muscular dystrophy (DMD) in this patient are the cutaneous manifestations and muscle tenderness. In addition, in children with DMD, symptoms and signs of muscle weakness are observed during infancy and certainly by the age of 3. By the age of 6 these children usually have muscle contractures and lordotic posture when walking. Furthermore, serum creatine kinase would be much higher (>15000 U/L) in DMD.
→ In Guillain-Barré syndrome (GBS) weakness usually begins in the lower limbs and progresses to the trunk, upper limbs and bulbar muscles. In addition, unlike the case of our patient, the weakness of GBS affects both proximal as well as distal muscles. Reflexes are either reduced or absent in this disorder and sensory complaints, in the form of paraesthesia, are common. Both motor and sensory conduction velocities are reduced in GBS. Cutaneous symptoms do not accompany this disorder and creatine kinase is normal.
→ Although any muscle can be affected, the weakness of Myasthenia gravis (MG) characteristically affects the ocular and bulbar muscles presenting as diplopia and dysphagia, respectively. In addition, MG has no known cutaneous manifestations. The typical electromyography finding of MG is the decremental response to repeated nerve stimulation. Furthermore, the weakness of MG waxes and wanes and is usually more marked towards the end of the day.
Key point:
A 16-year-old male is brought to your office by his mother for “stomachaches.” On the review of systems he also complains of headaches, occasional bedwetting, and trouble sleeping. His examination is within normal limits. His mother says that he is often in the nurse’s office at school, and doesn’t seem to have any friends. When you discuss these problems with him, he admits to being teased and called names at school.
Which one of the following would be most appropriate?
Correct Answer C:
Childhood bullying has potentially serious implications for bullies and their targets. The target children are typically quiet and sensitive, and may be perceived to be weak and different. Children who say they are being bullied must be believed and reassured that they have done the right thing in acknowledging the problem. Parents should be advised to discuss the situation with school personnel.
Bullying is extremely difficult to resolve. Confronting bullies and expecting victims to conform are not successful approaches. The presenting symptoms are not temporary, and in fact can progress to more serious problems such as suicide, substance abuse, and victim-to bully transformation. These are not signs or symptoms of thyroid disease.
The Olweus Bullying Prevention Program developed in Norway is a well documented, effective program for reducing bullying among elementary and middle-school students by altering social norms and by changing school responses to bullying incidents, including efforts to protect and support victims.
Students who have been bullied regularly are more likely to carry weapons to school, be in frequent fights, and eventually be injured.
A 7-year-old boy was brought to the hospital because of pain and swelling of his right knee and right wrist. His mother thought he was 'slightly feverish' and gave him Tylenol. The mother relates that a few days ago first his right ankle and then left wrist were similarly affected but the swelling and pain subsided over several days. About a month ago the patient had sore throat for which he received an antibiotic.
On examination, the patient was not pale, jaundiced, or cyanosed and he was febrile. His heart rate was 106 beats per minute, respiratory rate 22 resp/min and blood pressure was within normal range. The right knee and right wrist appeared red and swollen. All other joints were normal. The rest of the examination was unremarkable. Acute rheumatic fever was suspected and a couple of investigations were ordered. Anti-streptolysin O titer was positive.
In addition to the above clinical presentation which of the following would establish this diagnosis according to the revised Duckett Jones criteria?
Correct Answer B:
Rheumatic fever is one of the suppurative complications of group A streptococcal infection of the pharynx. Although there is a dramatic decline in its incidence in developed countries, rheumatic fever is still a significant problem because of its grave effects on the heart. Because of absence of any pathognomonic clinical or laboratory features for rheumatic fever, the revised Jones criteria are used for its diagnosis. The diagnosis can be established when a patient fulfills two major criteria or one major and two minor criteria plus an absolute requirement for evidence of recent group A streptococcal (GAS) infection. The major criteria include polyarthritis, carditis, subcutaneous nodules, erythema marginatum and chorea. The minor criteria include arthralgia, fever, high ESR, high C-reactive protein and prolonged PR interval. Noteworthy is the fact that these criteria should be used for the diagnosis of the initial acute attack only and not for recurrences.
This patient fulfills one major criterion (polyarthritis) and one minor criterion (fever; note that we cannot use arthralgia). An additional minor criterion is required to establish the diagnosis of acute rheumatic fever. High ESR (choice B) would thus be sufficient to establish the diagnosis of acute rheumatic fever.
→ Positive antistreptolysin O titer (choice A) is sufficient evidence for recent GAS infection. Positive throat culture will only strengthen this evidence and is not in itself a required criterion for diagnosis of acute rheumatic fever.
→ Mitral stenosis causing a rumbling mid-diastolic apical murmur (choice C) is a known complication of rheumatic heart disease. However, mitral stenosis usually develops years or decades after an acute attack of rheumatic fever. For this reason mitral stenosis does not signify acute carditis, a major criterion of diagnosis of acute rheumatic fever.
→ In contrast to mitral stenosis, mitral regurgitation (indicated by apical systolic murmur) might occur during an acute attack of rheumatic fever and is an evidence of acute carditis. For mitral regurgitation to be accepted as evidence of acute carditis, its murmur must be detected clinically. Subclinical or echocardiographically elicited systolic apical murmur (choice D) is so far not accepted as a conclusive evidence of rheumatic carditis.
→ The only erythematous rash (choice E) accepted as a criterion for diagnosis of rheumatic fever is erythema marginatum which occurs in the form of erythematous, serpiginous macular lesions with pale centers. It occurs primarily on the trunk and spares the face. Diffuse rash on the neck with flushed cheeks is characteristic of scarlet fever and not rheumatic fever.
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