Ray Boyapati

Seasonal recurrence of food bolus obstruction in eosinophilic esophagitis

Eosinophilic esophagitis (EoE) is a newly recognised condition that is apparently increasing in prevalence, and the aetiology is poorly understood. The role of aeroallergens in EoE is controversial, given the success of dietary therapy. Massive aeroallergen exposure leading to food bolus obstruction events (FBOE) has been described, and the diagnosis of EoE by esophageal biopsy noted to be more common in the pollen season according to previous case series.

AIMS65: A promising upper gastrointestinal bleeding risk score but further validation required

A novel upper gastrointestinal bleeding risk stratification score (AIMS65) has recently been developed and validated. It has advantages over existing risk scores including being easy to remember and lack of subjectivity in calculation. We comment on a recent study that has cast doubt on the applicability of AIMS65 in the peptic ulcer disease population. Although promising, further studies are required to evaluate the validity of AIMS65 in various populations.

Hepatobiliary and pancreatic: Systemic mastocytosis with portal hypertension

Mastocytosis is a rare disease characterised by the excessive accumulation of mast cells in one or more tissues. The most common form is mastocytosis limited to the skin (cutaneous mastocytosis). This often results in pruritus after skin irritation or changes in temperature. Another form is systemic mastocytosis where pathological mast cells infiltrate a variety of organs that may or may not include the skin. The extracutaneous sites usually include the bone marrow but may also include the liver and small bowel. Symptoms are highly variable but may be related to mediator release with flushing, syncope, nausea, vomiting, diarrhea and fatigue. Mast cell infiltration of the liver can also cause changes in liver function tests and there are case reports of portal hypertension. Higher levels of histamine can also cause duodenal ulceration. Blood tests may reveal an eosinophilia, monocytosis or elevated levels of tryptase. At present, there is no curative therapy for systemic mastocytosis. Symptomatic measures include avoidance of “triggers” including medication, lying down for light-headedness and epinephrine for symptoms of anaphylaxis. In the patient described below, an aggressive form of systemic mastocytosis was associated with portal hypertension and ascites. The patient was an 80 year old woman who was investigated because of a three month history of increasing abdominal distension and weight loss. There was no history of liver disease. On physical examination, the only abnormality was prominent ascites. Blood tests revealed a normal serum bilirubin and transaminase levels but there was a minor elevation of alkaline phosphatase (193 U/L) and gammaglutamyl transpeptidase (74 U/L). Her serum albumin was low at 22 g/L and she had a marginal elevation of INR (1.5). The acidic fluid was negative for malignant cells but she had a serum-ascites albumin gradient of 14 g/L indicating portal hypertension. A computed tomography scan showed an enlarged liver and spleen without features of cirrhosis as well as enlarged retroperitoneal lymph nodes up to 1.0 cm in diameter (Figure 1a, arrow). A positron emission tomography scan showed increased activity in the spleen and diffuse uptake in the bone marrow (Figure 1b, arrows). A bone marrow biopsy was reported as hypercellular and fibrotic without malignancy while fine-needle aspiration of retroperitoneal lymph nodes was not diagnostic. A liver biopsy taken at laparoscopy revealed an infiltrate of mast cells in the portal tract and sinusoids (Figure 1c) that were positive for c-kit (Figure 1d). The diagnosis was that of an aggressive form of systemic mastocytosis that is usually associated with a poor prognosis. Almost all patients with systemic mastocytosis have c-kit mutations but, unlike gastrointestinal stromal tumours, the majority of patients are resistant to imatinib therapy.

Hepatobiliary and pancreatic: Systemic mastocytosis with portal hypertension: Education and imaging

Mastocytosis is a rare disease characterised by the excessive accumulation of mast cells in one or more tissues. The most common form is mastocytosis limited to the skin (cutaneous mastocytosis). This often results in pruritus after skin irritation or changes in temperature. Another form is systemic mastocytosis where pathological mast cells infiltrate a variety of organs that may or may not include the skin. The extracutaneous sites usually include the bone marrow but may also include the liver and small bowel. Symptoms are highly variable but may be related to mediator release with flushing, syncope, nausea, vomiting, diarrhea and fatigue. Mast cell infiltration of the liver can also cause changes in liver function tests and there are case reports of portal hypertension. Higher levels of histamine can also cause duodenal ulceration. Blood tests may reveal an eosinophilia, monocytosis or elevated levels of tryptase. At present, there is no curative therapy for systemic mastocytosis. Symptomatic measures include avoidance of “triggers” including medication, lying down for light-headedness and epinephrine for symptoms of anaphylaxis. In the patient described below, an aggressive form of systemic mastocytosis was associated with portal hypertension and ascites. The patient was an 80 year old woman who was investigated because of a three month history of increasing abdominal distension and weight loss. There was no history of liver disease. On physical examination, the only abnormality was prominent ascites. Blood tests revealed a normal serum bilirubin and transaminase levels but there was a minor elevation of alkaline phosphatase (193 U/L) and gammaglutamyl transpeptidase (74 U/L). Her serum albumin was low at 22 g/L and she had a marginal elevation of INR (1.5). The acidic fluid was negative for malignant cells but she had a serum-ascites albumin gradient of 14 g/L indicating portal hypertension. A computed tomography scan showed an enlarged liver and spleen without features of cirrhosis as well as enlarged retroperitoneal lymph nodes up to 1.0 cm in diameter (Figure 1a, arrow). A positron emission tomography scan showed increased activity in the spleen and diffuse uptake in the bone marrow (Figure 1b, arrows). A bone marrow biopsy was reported as hypercellular and fibrotic without malignancy while fine-needle aspiration of retroperitoneal lymph nodes was not diagnostic. A liver biopsy taken at laparoscopy revealed an infiltrate of mast cells in the portal tract and sinusoids (Figure 1c) that were positive for c-kit (Figure 1d). The diagnosis was that of an aggressive form of systemic mastocytosis that is usually associated with a poor prognosis. Almost all patients with systemic mastocytosis have c-kit mutations but, unlike gastrointestinal stromal tumours, the majority of patients are resistant to imatinib therapy.

Education and Imaging. Hepatobiliary and pancreatic: systemic mastocytosis with portal hypertension.

Mastocytosis is a rare disease characterised by the excessive accumulation of mast cells in one or more tissues. The most common form is mastocytosis limited to the skin (cutaneous mastocytosis). This often results in pruritus after skin irritation or changes in temperature. Another form is systemic mastocytosis where pathological mast cells infiltrate a variety of organs that may or may not include the skin. The extracutaneous sites usually include the bone marrow but may also include the liver and small bowel. Symptoms are highly variable but may be related to mediator release with flushing, syncope, nausea, vomiting, diarrhea and fatigue. Mast cell infiltration of the liver can also cause changes in liver function tests and there are case reports of portal hypertension. Higher levels of histamine can also cause duodenal ulceration. Blood tests may reveal an eosinophilia, monocytosis or elevated levels of tryptase. At present, there is no curative therapy for systemic mastocytosis. Symptomatic measures include avoidance of “triggers” including medication, lying down for light-headedness and epinephrine for symptoms of anaphylaxis. In the patient described below, an aggressive form of systemic mastocytosis was associated with portal hypertension and ascites. The patient was an 80 year old woman who was investigated because of a three month history of increasing abdominal distension and weight loss. There was no history of liver disease. On physical examination, the only abnormality was prominent ascites. Blood tests revealed a normal serum bilirubin and transaminase levels but there was a minor elevation of alkaline phosphatase (193 U/L) and gammaglutamyl transpeptidase (74 U/L). Her serum albumin was low at 22 g/L and she had a marginal elevation of INR (1.5). The acidic fluid was negative for malignant cells but she had a serum-ascites albumin gradient of 14 g/L indicating portal hypertension. A computed tomography scan showed an enlarged liver and spleen without features of cirrhosis as well as enlarged retroperitoneal lymph nodes up to 1.0 cm in diameter (Figure 1a, arrow). A positron emission tomography scan showed increased activity in the spleen and diffuse uptake in the bone marrow (Figure 1b, arrows). A bone marrow biopsy was reported as hypercellular and fibrotic without malignancy while fine-needle aspiration of retroperitoneal lymph nodes was not diagnostic. A liver biopsy taken at laparoscopy revealed an infiltrate of mast cells in the portal tract and sinusoids (Figure 1c) that were positive for c-kit (Figure 1d). The diagnosis was that of an aggressive form of systemic mastocytosis that is usually associated with a poor prognosis. Almost all patients with systemic mastocytosis have c-kit mutations but, unlike gastrointestinal stromal tumours, the majority of patients are resistant to imatinib therapy.

To TOE or not to TOE? That is the question in patients with portal hypertension and varices

We read with interest your recently published review by Cardenas and Gines on patients with cirrhosis, awaiting liver transplantation,1 with an emphasis on the high morbidity and mortality with variceal bleeding. Such patients may require a transoesophageal echocardiogram (TOE) to assess cardiorespiratory abnormalities potentially precluding a transplant, as well as for intraoperative haemodynamic monitoring and for investigation for endocarditis. However, the safety of performing a TOE in such patients is debated by clinicians due to the perceived risk of postprocedural bleeding.2 Three small studies have analysed post-TOE bleeding complications in patients with varices, having reported no bleeding in 26, 14 and 23 patients.3–5 We hypothesised that direct external trauma by the echocardiographic probe is unlikely to cause significant bleeding, as variceal bleeding is primarily caused by wall tension, a function of increasing variceal diameter and internal pressure.6 We therefore audited post-TOE bleeding rates at an Australian liver transplant referral centre in patients who had both varices or portal hypertension and a TOE based on International Classification of Diseases 10 coding from 1995 …

Young woman with recurrent vomiting associated with weight loss

A 39-year-old woman presented with an 18-month history of episodic severe nausea, vomiting, diarrhoea and 30 kg weight loss requiring multiple hospital admissions. Her past medical history included hypothyroidism, migraines and lower back pain for which she used paracetamol, codeine and intermittent diclofenac up to 150 mg per day for up to a week during exacerbations. C reactive protein and erythrocyte sedimentation rate were raised up to 89 mg/l and 80 mm/h respectively during some of these admissions. She was iron, vitamin B12 and …