Normal Sinus Rhythm

A 50 Year old lady, newly diagnosed case of hypertension visits her general physician for a routine checkup. Her blood pressure is well controlled on Tab Enalapril 5 mg once daily. She has no active complains. Her BP is 110/80 mmHg and the rest of the  examination is unremarkable. Her ECG is shown below:

What is the rhythm shown in the ECG?

Answer: Normal Sinus Rhythm

Case Discussion:

A 7 Year Old Boy With Difficulty Standing Up And Delayed Milestones

This 7-year-old boy presented with history of difficulty in standing up and had delayed motor mile stones.

1. What is the sign called which is shown in the picture above?
2. What is the most probable diagnosis?
3. What is the prognosis of this disease?
4. Which cardiac problem is likely in such children?
5. Why males are exclusively affected?

Answers And Discussion:

1. What is the sign called which is shown in the picture above?

Answer: This sign is called as Gower’s sign. (classical climbing over one’s own legs to stand up from sitting posture is known as Gower's sign)

2. What is the most probable diagnosis?

Answer:  The most probable diagnosis is Duchenne muscular dystrophy (DMD).

Presence of prominent calf muscles in a boy with classical Gower’s sign and history of delayed motor mile stones is very much suggestive of Duchenne muscular dystrophy. Presence of raised CPK and absence of dystrophin in muscle biopsy would confirm the diagnosis.

It is initially characterized by delayed motor development in the child. Later on other characteristic features of this disease as calf muscle hypetrophy, Waddling gait (Trendelenburg gait) and classical climbing over one’s own legs (Gower’s sign) become apparent. Gower’s sign is seen by 3 years and

is fully manifested by 6 years. Along with the weakness of the muscles in lower limbs there is also weakness of the shoulder girdle muscles in these children. Pain is usually not associated with this weakness. Mental retardation is usually found to be associated with this disease. Hypertrophy is observed in tongue and the muscles of forearm in addition to the obvious hypertrophy of calf muscles. Though they appear hypertrophied but are actually weak in strength.Similarly, intellectual impairment occurs in all patients although only 20-30% have IQ less than 70.

CPK is raised classically and muscle biopsy reveals degeneration of muscle. If CPK levels are high in a child suspected to suffer from DMD then there is high probability of DMD. But if the CPK levels are normal then it definitely excludes the possibility of DMD in the child. Thus high level of CPK increases the possibility of DMD but the final confirmation of the diagnosis is possible only if the muscle biopsy reveals too low level of dystrophin in the muscle.

3. What is the prognosis of this disease?

Answer: This disease has a relentless progressive course. It ultimately results in bed ridden state of the child. Child in later stage is even unable to comb his hairs. Death usually occurs by about 18-20 years. The cause of death may be respiratory failure in sleep, intractable congestive cardiac failure, pneumonia, aspiration or airway obstruction.

Management

Maintenance of good nutrition and physiotherapy are the mainstays of its management. Steroids have no role in its management. 

Myoblast transfer is the latest technique being tried for its management. Its success is yet not established. In it myoblasts from the normal muscles of close but unaffected relatives usually the father is cultured in vitro. It is then injected in the dystrophic muscle. Simultaneously, the patient is immunosuppressed to prevent the rejection of the graft.

4. Which cardiac problem is likely in such children?

Answer: Cardiac assessment is important in these patients as cardiomyopathy is usually associated with it. It is a constant feature of DMD.

5. Why males are exclusively affected?

Answer: This disorder is classically transmitted as an X-linked recessive trait. So, it

is mostly seen in boys.

Treatment Options For A 78 year Old Woman With Pain In Her Knee Joint

A 78-year-old woman with mild renal insufficiency complains of pain in her knee joints on walking. The pain interferes with her day-to-day activities and is relieved by rest. There is no redness or swelling. There is minimal joint effusion.
An x-ray of the knee shows osteophytes and asymmetric loss of joint space and is shown below:

ESR and white blood cell count are normal.

Which of the following is the best initial management of this patient?
A. Naproxen
B. Indomethacin
C. Intra-articular corticosteroids
D. Acetaminophen
E. Total arthroplasty

Answer And Discussion:

Pathogenesis In A Patient With Thrombotic Thrombocytopenic Purpura

A 38-year-old woman presents with a 3-day history of fever and confusion. She was previously healthy and is taking no medications. She has not had diarrhea or rectal bleeding. She has a temperature of 38°C (100.4°F) and a blood pressure of 145/85. Splenomegaly is absent. She has no petechiae but does have evidence of early digital gangrene of the right second finger. Except for confusion the neurological examination is normal. Her laboratory studies reveal the following:
Hemoglobin: 8.7
Platelet count: 25,000
Peripheral smear: numerous fragmented RBCs, few platelets
LDH 562 (normal <180)
Creatinine: 2.7
Liver enzymes: normal
Prothrombin time/PTT/fibrinogen level: normal

What is the most likely pathogenesis of her condition?

A. Disseminated intravascular coagulation
B. Antiplatelet antibodies
C. Failure to cleave von Willebrand factor multimers
D. Verotoxin-induced endothelial damage
E. Cirrhosis with sequestration of erythrocytes and platelets in the spleen

Answer And Discussion:

A 5 Year Old Child Presents With Mild Fever And A Rash

A 5 year-old child is brought to the clinic with presenting complaints of mild fever and a rash mainly on the trunk with some lesions in the mouth. Rash was associated with pruritis and itching.
The appearance of rash is shown in the picture below:

The rash is in various stages including papules, vesicles and crusting that has appeared in crops.

1. What is the most probable diagnosis?
2. Which drugs are contraindicated in it and why?
3. Which complications can occur in this disease?
4. Which drug can help, if given in early phase of disease?
5. What can be done, if an un vaccinated adolescent comes in contact with this patient?

Answers And Discussion:

A 3 Year Old Girl With A Foreign Body In The Ear

A 3-year-old girl is brought by her parents to an urgent care facility after a day of crying, irritability, scant otorrhea, and frequent pulling of her right ear.
Otoscopy reveals an erythematous, swollen external auditory canal (EAC) where a bead is wedged (Picture shown below). The patient was referred to an otolaryngologist and the bead is removed
using an operating microscope for visualization.

Case Discussion:
Foreign Body In The Ear:

Introduction: Ear Foreign Bodiess are commonly seen in children ages 1 to 6 years. Most common FBs in children include:
~ Inanimate objects such as beads , cotton tips, paper, toy parts, crayons , eraser tips, food, or organic matter, including sand, sticks, and stones as well as sometimes insects.

Pathogenesis: Pathogenesis includes some of the key elements of otitis externa :