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Benign intrahepatic cholestasis (BRIC) is a rare genetic disorder characterized by episodic cholestasis. Each episode is characterized by repeated episodes of jaundice, intense pruritis last for weeks to months with complete remission. Although each episode is associated with significant morbidity, progressive liver injury and cirrhosis do not occur. In recent studies, few patients progressed to Progressive familial intrahepatic cholestasis. Here we report a case of 19 years boy with benign intrahepatic cholestasis due to an undulant course terminated by plasma exchange.

Presentation of severe pain syndromes prior to onset of motor weakness is an uncommon but documented finding in patients with Guillain-Barré syndrome (GBS). Sciatica in GBS is a difficult diagnosis when patients present with acute radiculopathy caused by herniated disc or spondylolysis. A middle-aged woman was admitted for severe low back pain, symptomatic hyponatraemia, vomiting and constipation. On further investigation, she was diagnosed with radiculopathy, and appropriate treatment was initiated. Brief symptomatic improvement was followed by new-onset weakness in lower limbs, which progressed to involve upper limbs and right extraocular muscles. With progressive, ascending, new-onset motor and sensory deficits and laboratory evidence of demyelination by Nerve Conduction Study, a diagnosis of variant GBS was made. She was treated with intravenous immunoglobulin 2 g/kg over 5 days. The presentation of severe low back pain that was masking an existing aetiology and possible dysautonomia and the unilateral right extraocular muscles instead of bilateral make our case unique and rare.

Acute flaccid paralysis is a neuromuscular emergency characterized by rapidly worsening weakness that evolves quickly to cause diaphragmatic failure. The challenge for the treating physician is to stabilize the patient, generate the differential diagnosis and determine the management; all in quick time. Neurotoxic snake bites have inadequate signs of inflammation and are easily missed. Myasthenic crisis, on the other hand, could be the first sign of myasthenia gravis in up to 20% of patients. Both present with acute respiratory failure and inadequate history. Two of our patients presented with similar clinical picture, and received polyvalent anti-snake venom obtained from hyperimmunised horses (Equus caballus). Both tested positive for anti-acetyl choline receptor antibody. After recovery, both patients narrated a history suggestive of neurotoxic envenomation. We later discovered that patients, who are exposed to polyvalent anti-snake venom (Equus caballus) prior to radioimmunoassay, demonstrate high titers of Anti-AChR Ab in their serum erroneously.

Accurate identification of low-risk patients with acute pulmonary embolism (PE) who may be eligible for outpatient treatment or early discharge can have substantial cost-saving benefit. The purpose of this study was to derive and validate a prediction model to effectively identify patients with PE at low risk of short-term mortality, right ventricular dysfunction, and other nonfatal outcomes. This study analyzed data from 400 consecutive patients with acute PE. We derived and internally validated our prediction rule based on clinically significant variables that are routinely available at initial examination and that were categorized and weighted using coefficients in the multivariate logistic regression. The model was externally validated in an independent cohort of 82 patients. The final model (HOPPE score) consisted of 5 categorized patient variables (1, 2, or 3 points, respectively): systolic blood pressure (120, 100 to 119,99 mm Hg), diastolic blood pressure (80, 65 to 79,64 mm Hg), heart rate (80, 81 to 100,101 beats/min), arterial partial pressure of oxygen (80, 60 to 79,59 mm Hg), and modified electrocardiographic score (2, 2 to 4,4). The 30-day mortality rates were 0% in low risk (0 to 6 points), 7.5% to 8.5% in intermediate risk (7 to 10), and 18.2% to 18.8% in high-risk patients (≥11) across the derivation and validation cohorts. In comparison with the previously validated PESI score, the HOPPE score had a higher discriminatory power (area under the curve 0.74 vs 0.85, p = 0.033) and significantly improved both the discrimination (integrated discrimination improvement, p = 0.002) and reclassification (net reclassification improvement, p = 0.003) of the model for short-term mortality. In conclusion, the HOPPE score accurately identifies acute patients with PE at low risk of short-term mortality, right ventricular dysfunction, and other nonfatal outcomes. Prospective validation of the prediction model is necessary before implementation in clinical practice.

A 43-year-old farmer presented with acute onset pneumonia, septicaemia and peripheral facial nerve palsy (left side). Burkholderia pseudomallei was isolated from the blood culture of the patient. The patient was successfully treated with intravenous meropenem and oral cotrimoxazole for 2 weeks followed by maintenance therapy with cotrimoxazole. The case is reported to increase awareness among the clinicians and microbiologists regarding melioidosis.