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Objective: To determine periextubation practices in extremely preterm infants (<28 weeks gestation). Design: A survey consisting of 13 questions related to weaning from mechanical ventilation, assessment of extubation readiness and postextubation respiratory support was developed and sent to clinical directors of level III NICUs in Australia, Canada, Ireland, New Zealand and USA. A descriptive analysis of the results was performed. Results: 112/162 (69%) units responded; 36% reported having a guideline (31%) or written protocol (5%) for ventilator weaning. Extubation readiness was assessed based on ventilatory settings (98%), blood gases (92%) and the presence of clinical stability (86%). Only 54% ensured that infants received caffeine ≤24 h prior to extubation. 16% of units systematically extubated infants on the premise that they passed a Spontaneous Breathing Test with a duration ranging from 3 min (25%) to more than 10 min (35%). Nasal continuous positive airway pressure was the most common type of respiratory support used (84%) followed by nasal intermittent positive pressure ventilation (55%) and high-flow nasal cannula (33%). Reintubation was mainly based on clinical judgement of the responsible physician (88%). There was a lack of consensus on the time frame for definition of extubation failure (EF), the majority proposing a period between 24 and 72 h; 43% believed that EF is an independent risk factor for increased mortality and morbidity. Conclusions: Periextubation practices vary considerably; decisions are frequently physician dependent and not evidence based. The definition of EF is variable and welldefined criteria for reintubation are rarely used. Highquality trials are required to inform guidelines and standardise periextubation practices. [ABSTRACT FROM AUTHOR]

Background Cowden syndrome (CS) is an autosomal dominant disorder characterised by macrocephaly, specific mucocutaneous features and predisposition to benign and malignant tumours. Detectable mutations in the PTEN gene account for 80-85% of cases. Methods/Results Here, the authors report a patient with macrocephaly and typical CS mucocutaneous features who developed dysplastic cerebellar gangliocytoma and two synchronous thyroid cancers of papillary and oncocytic type, in whom a germline 500-Kb deletion on chromosome 10q23 including PTEN was detected. Molecular characterisation of thyroid cancer led to the identification of the oncogenic BRAFV600E mutation in the papillary carcinoma. BRAFV600E has been proposed to cause cancer only in the presence of a tumour-suppressor mutation, which, in this case, could be the PTEN deletion. In the oncocytic carcinoma, a large deletion in the mitochondrial-DNA-encoded MTND1 was found, associated with respiratory complex I disassembly, which was subsequently shown to be a constitutional, de novo genetic lesion. Conclusions This is the first reported case of a patient with CS carrying constitutional deletions in both the nuclear and the mitochondrial genome that might help elucidate some aspects of CS pathogenesis. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 3-year-old boy with 4-day history of fever and diffuse itchy eruption on his face. His medical history include adenoidal hypertrophy wherein he received oral betamethasone sodium phosphate for ten days followed by budensonide nebuliser. The patient underwent physical examination and showed erythematous patches from his face down to his legs. The patient was diagnosed with erythema multiforme wherein he received oral antihistamine and antibiotic.

A case study is presented of a 2-year-old girl with a 3-month history of small painless papules, growing on the surface of an abdominal congenital melanocytic naevus (MN). Dermoscopic examination suggested molluscum contagiosum (MC), which is a common viral skin infection. It relates the occurrence of viral lesions on an MN with the Meyerson's phenomenon, which is an eczematous reaction occurring in MN.

The article presents a case study of a nine year old child who was referred to pediatric dermatology unit for a temporary walking impairment due to painful lesions on the soles of both his feet. Several laboratory tests such as erythrocyte sedimentation rate, serum reactive protein, and antinuclear antibody. The article discusses diagnosis of palmoplantar eccrine hidradenitis (PEH), pathogenesis of PEH, and erythematous violaceous papules in PEH.

The article presents a case study of a six-month-old infant with itching erythema. It informs that diffuse cutaneous mastocytosis (DCM) diagnosis revealed vesicle-bullous eruption, skin infiltration and dermographism. It also mentions that skin prick tests may induce an anaphilaxis in infants and tryptase levels were consistently high in the infant.

The article presents a case study of one month old girl who had a firm, sharply marginated yellowish plaque on the right mandibular region. On medical examination, it was found that she had developed giant congenital juvenile xanthogranuloma (JXG). It mentions that JXG is the most common form of non-Langerhans cell histiocytosis that is characterized by purple-red to yellow-brown papules. It also states that correct treatment is necessary in this case to avoid further harsher treatment.