Acute liver failure secondary to ABVD use

Hodgkin's lymphoma (HL) is a type of cancer originating in the lymph nodes. The preferred therapy for advanced HL is a combination of chemotherapies including doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD). ABVD has been standard therapy for advanced HL. It is generally considered as safe and rarely has been reported to cause acute liver failure. We present a case of 79-year-old woman with HL, who developed acute liver failure secondary to first cycle of ABVD chemotherapy.

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Recurrent invasive group A streptococcal infection with four-limb amputation in an immunocompetent child

We report a previously well paediatric patient with two distinct presentations of invasive group A streptococcus (GAS) infection resulting in significant morbidity. The first episode, following GAS pharyngitis, involved multiorgan dysfunction syndrome. This included cardiorespiratory and acute hepatorenal failure and purpura fulminans that eventually necessitated four-limb amputation. The second episode occurred 12 months later, from undetermined aetiology, and resulted in septic shock. Molecular analysis of the emm gene and PCR for Serum Opacity Factor revealed that the initial isolate was M Type 4 and sof gene positive while the second isolate was M Type 1 and sof gene negative. Immunological investigations, including CH50, quantitative IgA, IgM and IgG, and flow cytometry measuring lymphocyte subsets, and vaccine response to measles, mumps, rubella and pneumococcus were normal. This is the first report of recurrent bacteraemia from different strains of Streptococcus pyogenes infection in an apparently immunocompetent child.

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Primary renal angiosarcoma

An older male patient with a history of tachycardia treated with atenolol presented to an outside hospital on 22 February 2017 with acute right flank pain. He had a CT scan which revealed a large right renal mass with acute haemorrhage. He was initially managed with interventional radiology guided embolism on 25 February 2017 due to the ongoing bleeding and haemodynamic instability. He was then transferred to our institution. He underwent right radical nephrectomy on 13 March 2017. His pathology revealed a 12.5x6x4.5 cm mass consistent with angiosarcoma of the right kidney with negative margins. Final pathology was pT2b with extension of the mass into the renal vein and perirenal adipose tissue. He was discharged soon after surgery. He was recommended to undergo adjuvant chemotherapy.

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Extensive Mongolian spots and normocephaly: an uncommon presentation of infantile Sandhoffs disease

Description 

A 10-month-old male infant was brought to us with developmental stagnation since 5 months of age. He attained age-appropriate developmental milestones until 5 months of age, however over the next 2 months, he did not gain any new milestones followed by subsequent developmental regression in the form of loss of neck holding ability, social smile, mother regard, visual fixation and cooing. He was a first born to non-consanguineous parents, at term by caesarean delivery (due to non-progression of labour). The antenatal and perinatal periods were normal. There was no history of seizures, exaggerated startle response and extrapyramidal symptoms. The family history was unremarkable. On examination, he had normal head size (46.2 cm, 50th centiles), dysmorphic facial features (flat nasal bridge, hypertelorism, thick upper lip and upturned nose), bilateral cherry-red spots, extensive Mongolian spots (figure 1) and hepatomegaly. He also had generalised hypotonia, hyperactive muscle stretch reflexes and bilateral...

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Platypnoea-orthodeoxia syndrome exacerbated by kyphosis progression

This is a case of an 86-year-old woman with gradually progressive dyspnoea and hypoxaemia that occurred after a cardiac surgery. It was underdiagnosed for several years, but diagnosis was triggered by the finding of hypoxaemia even during supplemental oxygen administration when in the upright position, such as when taking a shower, that rapidly improved when the patient returned to the supine position. A thorough workup disclosed platypnoea–orthodeoxia syndrome (POS) associated with right-to-left shunting through a patent foramen ovale (PFO). Percutaneous closure of the PFO was performed. After treatment, the patient's arterial oxygen saturation gradually recovered to 98% on room air while she was in the sitting position and her symptoms disappeared. Reviewing this case retrospectively, we determined that the deviation of the spine with kyphosis progression had apparently proceeded as POS worsened over time. We therefore hypothesised that kyphosis progression had played a major role in the POS progression.

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Liver failure associated with mahogany seed extract consumption

A 45-year-old woman presented to the emergency department with jaundice of 2 weeks' duration. This was associated with mahogany seed extract (Skyfruit supplement) consumption for 6 months prior to admission. Examination was normal apart from scleral icterus and grade 2 encephalopathy. Liver function tests showed a hepatocellular pattern of derangement: alanine transaminase, 1267 U/L (10–36); aspartatetransaminase, 1255 U/L (10–30); alkaline phosphatase, 124 U/L (22–104); bilirubin, 258 µmol/L (3–21) with a prolonged prothrombin time of 16.8 s (9.2–11.0). Viral hepatitis work-up was largely unremarkable and liver biopsy showed moderate inflammatory infiltrates (mostly lymphocytic with scattered eosinophils) in the periportal region and lobule with bridging necrosis, favouring drug-induced liver injury. Withdrawal of the drug resulted in normalisation of liver function.

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Posterior shoulder dislocation: its worth another look

A 40-year-old woman presented to the emergency department with a painful left shoulder following a fall from a mountain bike. A radiograph of the clavicle and shoulder was carried out. A diagnosis of fractured left clavicle was made, which was treated conservatively in a sling. On day 3, she was reviewed in the fracture clinic and was found to still be in pain with reduced movement of the shoulder joint. A repeat radiograph of the shoulder joint showed a posterior dislocation. This required a closed reduction under general anaesthesia.

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