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The article focuses on double gibbus of two-year-old girl that results due to spinal tuberculosis (TB). The clinical symptoms of spinal TB in a child are often insidious and include back pain, fever, paraparesis, sensory disturbance and bowel and bladder dysfunction. Histological proof of TB spine by isolation or culture of Mycobacterium tuberculosis is obtained only in a few patients who undergo surgery. In the majority of the cases of TB spine, the diagnosis is confirmed on characteristic Magnetic Resonance Imaging findings along with other positive findings namely, insidious clinical history of fever and anorexia; positive Mantoux test; suggestive chest radiography/ CT scan chest findings or a positive response to anti-TB drug therapy.

The article presents a case of a 4.5 year old boy who was suffering from skeletal dysplasia. On examination, the child was grossly stunted. The height was 78 cm, weight 10 kg, and the head circumference 48 cm. The height and weight were markedly below the fifth percentile. The upper segment: lower segment ratio was 1.34:1 indicating proportionate short stature. On abdominal examination, the spleen was enlarged 13 cm below the left costal margin and the liver was palpable 5 cm below the right costal margin, both were firm and non-tender. Fundoscopy revealed bilateral papilloedema with no evidence of secondary optic atrophy. The rest of the systemic examination was normal.

Pulmonary manifestations are seldom recognized as symptoms of storage disorders. The report describes the diagnostic journey in a 30-month-old male infant, born of a third-degree consanguineous marriage referred to our institute as severe persistent asthma. History revealed that the child had progressively worsening breathlessness and persistent dry cough not associated with fever but accompanied by weight loss. On physical examination, there was growth failure, respiratory distress, clubbing, hepatosplenomegaly, and occasional rhonchi. Blood gas revealed hypoxemia which improved with oxygen administration. Plain X-rays and high-resolution computed tomography of the chest showed perihilar alveolar infiltrates and patchy consolidation. The clinicoradiological features did not support a diagnosis of asthma but favored interstitial lung disease (ILD). Bronchoalveolar lavage was performed as a first-tier investigation. It showed periodic acid-Schiff-negative foamy macrophages. The clues of consanguinity, visceromegaly, ILD, and foamy macrophages in the bronchoalveolar fluid prompted consideration of lysosomal storage disorders as the likely etiology. Gaucher disease and Niemann-Pick disease A/B were ruled out by enzyme estimation. Niemann-Pick disease type C was suspected and confirmed by detecting a homozygous mutation in the NPC2 gene. This case serves to caution physicians against labeling breathlessness in every toddler as asthma. It emphasizes the importance of searching for tell-tale signs such as clubbing and extrapulmonary clues which point to a systemic disease such as lysosomal storage disorders as a primary etiology of chronic respiratory symptoms. [ABSTRACT FROM AUTHOR]

The article provides updates on issues related to generic drugs in India as of April 2019. Topics covered include the enactment of The Drug Price Competition and Patent Term Restoration Act on September 24, 1984 in the 98th U.S. Congress, the launch of Jan Aushadhi initiative by the Department of Pharmaceuticals in 2008, and amendment of Rule 65 (11A) of the Drugs and Cosmetics Act considered by the Drugs Technical Advisory Board of India in May 2016.

The article presents a case study of a 3-month-old infant with progressing breathlessness, fever and feeding difficulty among other conditions. Examinations showed skeletal abnormalities, enlarged kidneys, and pulmonary hypertension, and the infant had a suggested diagnosis of skeletal ciliopathies like short rib thoracic dysplasia (SRTD). The patient was given supplemental oxygen and oral nifedipine and sildenafil among other treatments and was discharged after 33 days on nasal oxygen.

Deep vein thrombosis and pulmonary thromboembolism are rare and life threatening emergencies in children. We report an 11-year old female who presented with acute complaints of high grade fever, pain in the left thigh and inability to walk and breathlessness since 6 days. On physical examination, there was a diffuse tender swelling of the left thigh, tachypnea, tachycardia with hyperdynamic precordium and bilateral basal crepitations. Ultrasonography and venous doppler of lower limbs showed mild effusion of left hip joint and thrombus in the left common femoral vein and left external iliac vein suggesting a diagnosis of septic arthritis with thrombophlebitis. The tachypnea and tachycardia which was out of proportion to fever and crepitations on auscultation prompted suspicion of an embolic phenomenon. Radiograph of the chest revealed multiple wedge shaped opacities in the right middle zone and lower zone suggestive of pulmonary embolism and left lower zone consolidation. For corroboration, computed tomography pulmonary angiography and computed tomography of abdomen was performed which showed pulmonary thromboembolism and deep venous thrombosis extending up to infrarenal inferior vena cava. On further workup, magnetic resonance imaging of hips showed left femoral osteomyelitis and multiple intramuscular abscesses in the muscles around the hip joint. Blood culture grew methicillin resistant Staphylococcus aureus. Antibiotics were changed according to culture sensitivity and there was a dramatic response. After four weeks of anticoagulation and antibiotics the child became asymptomatic and thrombus resolved. Thus, it is crucial to consider methicillin resistant Staphylococcus aureus infection as an important infection when we encounter such a clinical scenario. This case report highlights an unusual and potentially life threatening presentation of a virulent strain of a common pathogen, which when diagnosed was completely amenable to treatment. [ABSTRACT FROM AUTHOR]