The action of modern emergency care for patients with AP, on the one hand, requires a wait-and-see period, which is unacceptable in cases of rapidly progressing inflammation. On the other hand, it uses methods that do not take into account the features of the disease mechanisms. All this creates conditions for an excess of complications and treatment failures. The accumulated facts on this problem indicate the need to revise the principles of treatment. Consequently, the most important initial step in this direction seems to be the correction of professional views in accordance with the classical provisions of medical science.
Pachydermoperiostosis, also known as Primary Hypertrophic Osteoarthropathy (PHO), is a rare genetic disorder. The three main features are: enlarged fingertips (clubbing), thickened facial skin (pachydermia), and excessive sweating (hyperhidrosis). PHO is characterized by problems with skin and bone growth. Patients with PHO usually have coarse facial features with oily, thick, grooved skin on the face, joint pain, enlarged fingertips and toes, and hyperhidrosis of the hands and feet. Symptoms vary individually; however, men generally present with more severe manifestations. X-rays can help check for features that are not noticeable to the naked eye. There are two genes that are associated with PHO: the HPGD gene, located on the long arm of chromosome 4 at 4q34.1, and the SLCO2A1 gene, located on the long arm of chromosome 3 at 3q22.1 - q22.2. Mutations in the HPGD gene are inherited in an autosomal recessive manner, and the condition is sometimes abbreviated as PHOAR1 or Touraine-Solente-Gole syndrome.
Introduction: Menarche, the onset of a girl's first menstrual cycle, often introduces menstrual taboos in certain eastern cultures. These taboos may manifest as social isolation, dietary restrictions, and exclusion from religious spaces, which can adversely affect health and promote gender inequality.Case history: A 10-year-old girl developed gastroenteritis while in cultural confinement after reaching menarche. Her parents strictly adhered to traditional customs that limited her interactions with the outside world and deprived her of healthy foods. They believed that her fatigue was a result of hormonal changes associated with menarche. Although her condition worsened over four days, they did not seek medical treatment. The child succumbed upon admission. The autopsy revealed extensive cyanosis in the left hand due to multiple thrombotic occlusions of the brachial vein. Microscopic examination confirmed the presence of brachial venous thrombi. The cause of death was determined to be Multiple Organ Dysfunction Syndrome (MODS) as a consequence of hypovolemic shock.Discussion: Key medico-legal issues in this case include parental negligence and failure to provide medical care under Section 308A of the Penal Code, potentially leading to criminal liability for negligence-related death under Section 298. Violations of the child's rights, protected by the Children and Young Persons Ordinance (CYPO) and the Protection of Children's Rights Act, form the legal framework for child protection in Sri Lanka. The ISD has interviewed family members and collected witness statements from neighbors and teachers, referring the case to the police for further investigation and notifying the National Child Protection Authority for an additional inquiry. Additionally, Sri Lanka is a signatory to the United Nations Convention on the Rights of the Child (UNCRC), which guarantees children's fundamental rights.
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