Stridor is not always croup: infantile haemangioma in the airway

Description

A 12-month-old boy presented with a history of respiratory distress and stridor since the first month of life. He had been repeatedly misdiagnosed as recurrent croup. The stridor was biphasic, with a more pronounced inspiratory component, and was exacerbated by agitation and supine positioning. He had a large posterior cervical haemangioma, whose extension had never been studied by imaging methods, and delayed growth. Flexible bronchoscopy showed a multilobulated subglottic haemangioma (SGH), occupying more than 70% of the tracheal lumen. MRI showed an angiomatous malformation with the epicentre at the hind head that extended inferiorly to the cervical planes, reaching the median line in retropharyngeal planes with inferior extension (figures 1 and 2). He underwent systemic and intralesional tracheal steroids injections with partial improvement and laser therapy at 5 years of age. Today he is an asymptomatic 14-year-old adolescent with normal growth and development.

Figure 1

Cervical...

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Toxic multinodular goitre: a surprising finding

Description

A 16-year-old healthy adolescent boy was referred to the paediatric endocrinology clinic because of multiple thyroid nodules detected by cervical ultrasound, in the context of cervical lymphadenopathies. There was no family history of thyroid disease. He denied recent infections, asthenia, weight loss, sweating, palpitations, mood or sleep disturbances, dysphagia or dysphonia. At physical examination, an enlarged, irregular and fibroelastic thyroid, with a predominant right lobe, was identified. The remaining examination was normal.

The analytical profile was thyroid stimulating hormone (TSH) <0.01 uUI/mL (normal 0.5–4.8 uUI/mL), free triiodothyronine (FT3) 7.27 pg/mL (normal 2.3–4.2 pg/mL) and free thyroxine (FT4) 2.02 ng/dL (normal 0.8–2.3 ng/dL). Thyroid antibodies were negative. Cervical ultrasound revealed an enlarged right thyroid lobe due to the presence of multiple mixed nodules with similar characteristics: the biggest one, 5 cm diameter, predominantly cystic with a peripheral ring of solid isoechoic parenchyma (figure 1A); the second characteristically mixed nodule, 2.2 cm diameter (

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Fever, bone pain and erectile dysfunction. Where is the cat?

Cat-scratch disease is due to Bartonella henselae and commonly presents as a localised papular lesion with regional lymphadenopathy. We report the case of a young man suffering general symptoms and dysautonomy characterised by an erectile dysfunction due to an invasive cat-scratch disease. He was successfully treated by tetracyclines during 3 weeks.

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Pulsus alternans: a visual clue to a grave disorder!

Description

A 15-year-old, previously healthy teenager who had fatigue, shortness of breath, vomiting, chest discomfort, lower extremity oedema and inability to lay flat for 3 weeks prior to her initial presentation. She was warm, well perfused with alternating strong and weak central as well as peripheral pulses and laterally displaced cardiac impulse. Her monitoring showed visual evidence of 'pulsus alternans'—alternating low and high amplitude waveforms on the arterial waveform and plethysmography (figure 1). Her echocardiography showed severely depressed left ventricular function with ejection fraction of 15%. In addition, sinus tachycardia and varying capnography waveform supported the diagnosis. She gradually worsened requiring intubation and mechanical ventilation. She was diagnosed with primary cardiomyopathy. She underwent the placement of ventricular assist device and now awaits heart transplant.

Figure 1

Monitor showing (A) sinus tachycardia on telemetry. (B) Plethysmography showing alternating low and high amplitude waves as indicated...

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Immunological efficacy of herbal medicines in prostate cancer patients treated by personalized peptide vaccine

Summary

This randomized phase II study investigated the immunological efficacy of herbal medicines (HMs) using Hochu-ekki-to and Keishi-bukuryo-gan in combination with personalized peptide vaccination (PPV) for castration-resistant prostate cancer (CRPC). Seventy patients with CRPC were assigned to two arms; PPV plus HMs or PPV alone. Two to four peptides were chosen from 31 peptides derived from cancer antigens for the eight-time subcutaneous injection of PPV according to the patient's human leukocyte antigen type and levels of antigen-specific immunoglobulin G (IgG) titer before PPV treatment. Peptide-specific cytotoxic T lymphocyte (CTL), IgG, regulatory T cells (Treg), monocytic myeloid-derived suppressor cells (Mo-MDSC), and interleukin-6 (IL-6) responses were measured before and at the 8th vaccination. Clinical outcomes were also analyzed. The combination therapy of PPV with HMs was well tolerated without severe adverse events. There was no significant change in antigen-specific IgG, CTL, Treg or clinical outcomes. The combination therapy of PPV with HMs stabilized the frequency of Mo-MDSC (1.91% to 1.92%, p = 0.96) and serum levels of IL-6 (19.2 pg/ml to 16.1 pg/ml, p = 0.63) during the treatment. On the other hand, the frequency of Mo-MDSC and levels of IL-6 in the PPV alone group were significantly increased (0.91% to 1.49% for Mo-MDSC and 9.2 pg/ml to 19.4 pg/ml for IL-6, respectively). These results suggested that the combined usage of HMs has the potential to prevent the immunosuppression induced by Mo-MDSC or IL-6 during the immunotherapy. More research is needed to validate the findings of the present study.

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Clinical features of cystatin A expression in patients with pancreatic ductal adenocarcinoma

Summary

Pancreatic ductal adenocarcinoma (PDAC) is the most lethal malignancy known, with an extremely poor prognosis due to the lack of an efficient diagnostic scheme and no radical treatment option, except surgery. Therefore, understanding the pathophysiology of, and finding a novel biomarker to detect, PDAC should be prioritized. We observed an increase in mRNA expression of the cysteine protease inhibitor cystatin A (CSTA) in CD4+T cells in peripheral blood cells of 9 patients with PDAC, compared with the expression in 7 healthy volunteers. Moreover, we confirmed significantly higher CSTA mRNA expression in a larger cohort of 41 patients with PDAC compared with that in 20 healthy volunteers. Correspondingly, the serum CSTA concentrations in 36 patients with PDAC were higher than those in 37 healthy volunteers, and this increase was correlated with PDAC clinical stage. Furthermore, the expression of CSTA and cathepsin B, which is a lysosomal cysteine protease inhibited by CSTA, was observed in tumor tissues and tumor-infiltrating immune cells in 20 surgically resected PDAC tissues by immunohistochemical staining. CSTA expression was detected in some tumor tissues and many tumor-infiltrating immune cells. Cathepsin B expression was also observed in most tumor tissues and tumor-infiltrating immune cells. In conclusion, CSTA and its substrate cathepsin B are involved in PDAC-related inflammation. The increment of CSTA expression in peripheral blood of patients with PDAC may have a potential role as a PDAC immunopathological biomarker.

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An Austrian view of the 2017 consensus for the treatment of early breast cancer

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