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EN
Carpal tunnel syndrome and benign recurrent intrahepatic cholestasis are rare conditions in childhood. Benign intrahepatic cholestasis is characterized by repeated self-limited attacks of cholestasis that can start at any age and last from weeks to months. The patients are asymptomatic between these attacks. We report a 16 year-old male patient with benign recurrent intrahepatic cholestasis who developed carpal tunnel syndrome during a cholestatic attack. He was admitted with complaints of jaundice, pruritus and pain, tingling and muscle weakness in both hands for 15 days. Nerve conduction studies revealed findings compatible with carpal tunnel syndrome. He was started on ursodeoxycholic acid, fat soluble vitamins and cholestyramine and cholestasis regressed after four weeks of therapy. With the improvement of cholestasis, the symptoms of carpal tunnel syndrome also disappeared. In conclusion, benign recurrent intrahepatic cholestasis can be a rare cause of carpal tunnel syndrome in childhood. We also advocate treating the underlying disease as an appropriate conservative treatment before surgery.
EN
The paper briefly discusses the process of meaning verbalization by children understood as the exponent of meaning shaping. The author, basing upon the consecutive stages of the development of child’s speech presented by Leon Kaczmarek, demonstrates the specific features to be found in each of the stages. The theoretical discussion was completed with a number of examples taken from the author’s own research activity. Additionally, the salience of culture conditioning in the process of gaining of one’s language competence was focused upon.
EN
The formation of the identity of child with the mental disability in the educational process is the second part of the paper, which purpose is the approximation of the fundamental truth about the person with mental disability. The realization of this truth is the task of the education. The task of the educator is to help to every child to discover and experience its dignity and to recognise its identity. The man experiences and develops himself through the relations with people and existential situations. The child with the mental disability can grow up like another children but it needs proper stimulations and good conditions to the comprehensive activity during the whole educational process. There is a very important thing to overcome the hindrances and difficulties in the educational process. The necessity of the overcoming is included in the nature of the man. The realization of this need has the influence on the proper mental activity of child. The children with the mental disability have the same psychological stages like another children. After childhood and adolescent period the child becomes adult. It is necessary to help to him in selecting of proper dress, behaviour, playing and activity in this age. The growing of independence is a very important process for the future identity. The fundamental role plays the family. If the participation in the social life completes the proper educational influence of the family, the child will experience abundance of its personality, value of life and happiness of its being.
5
Content available remote Ascites with no fluid in Morison’s pouch?
75%
EN
Omental cysts are the least common cystic lesions of the abdomen. Large intraabdominal cysts can mimic ascites. The incidence of 1 in 20000 pediatric hospital admissions makes them hard to diagnose. We report a three year-old boy with accidentally discovered intraperitoneal fluid collection on ultrasonography. The patient was scheduled for operative treatment with working diagnosis of ascites, but finding of no fluid in Morison’s pouch brought to massive cystic intraabdominal lesion as differential diagnosis. The cyst was emptied; altered parts of the greater omentum were completely excised. Final pathology confirmed lymphangioma. Explorative laparotomy led to correct diagnosis and proper surgical treatment.
EN
Spinal Muscular Atrophy (SMA) is a neuromuscular disease caused by degeneration of alphamotoneurons. It leads to progressive muscle atrophy and weakness, and, in more severe cases to a total loss of motor function, respiratory insufficiency and death. In November 2017, an update in the Standards of Care (SOC) in SMA was published. For detailed rehabilitation guidelines, the patients were divided into groups according to their functional state – „bedridden”, „sedentary” and „ambulatory”. We focus on the guidelines for physiotherapy and rehabilitation, more specifically on methods of functional assessment recommended for the youngest patients with SMA. For evaluation, it is suggested to use either CHOP-INTEND (The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders) or HINE (Hammersmith Infant Neurological Examination) that are both based on WHO (World Health Organization) milestones. Regular assessment is necessary both for the evaluation of the course of the disease, as well as to capture possible improvement through pharmacological treatment.
PL
Rdzeniowy zanik mięśni (SMA – ang. Spinal Muscular Atrophy) to choroba nerwowo-mięśniowa charakteryzująca się degeneracją motoneuronów alfa – komórek rogów przednich rdzenia kręgowego. Prowadzi to do postępującej atrofii i osłabienia mięśni, zaś w cięższych przypadkach do całkowitej utraty funkcji motorycznych, niewydolności oddechowej i zgonu. W listopadzie 2017 ukazała się aktualizacja standardów opieki (Standards of Care – SOC) w SMA. Istotne miejsce w opiece ma fizjoterapia oraz ocena stanu funkcjonalnego przy pomocy walidowanych skal. Dla wyznaczenia szczegółowych wytycznych dotyczących rehabilitacji pacjenci zostali podzieleni ze względu na ich stan funkcjonalny na „niesiedzących”, „siedzących” oraz „chodzących”. W aktualnym omówieniu prezentujemy metody oceny funkcjonalnej dla najmłodszych pacjentów z SMA. Do ewaluacji zalecane są skale CHOP-INTEND (The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders) oraz HINE (Hammersmith Infant Neurological Examination). Regularna ocena jest wskazana dla określenia przebiegu choroby oraz skuteczności potencjalnych nowatorskich terapii SMA.
PL
Celem artykułu jest wskazanie na fenomen świadomej bezdzietności jako jeden z elementów przemian w zakresie formowania rodziny, jakie dokonują się współcześnie. Dotychczasowe wzorce tworzenia i funkcjonowania rodziny i wysoka wartość przypisywana macierzyństwu są podstawą stereotypizacji osób bezdzietnych z wyboru jako egoistycznych oraz materialistycznych. Analiza wyników badań jakościowych przeprowadzonych wśród par, które nie chcą mieć dzieci, ma wskazać na wielość źródeł wyboru wynikających ze zmian społeczno-kulturowych w społeczeństwie ponowoczesnym. Skupiono się zatem na poznaniu praktyk dyskursywnych i zrozumieniu motywacji osób, które świadomie odrzucają rodzicielstwo oraz współczesnych trudnościach terminologicznych związanych z pojęciem rodziny.
EN
The aim of the article is to point to conscious childlessness phenomenon as one of the transformations in family formation that are occurring today, in modern society. Past patterns of family creating and functioning, and the high value attributed to motherhood, are the basis for the stereotyping of childfree people as selfish and materialistic. The analysis of the results of qualitative research among couples who do not want to have children aims to demonstrating the multiple sources of this choice which are due to socio-cultural changes in post-modern society. The focus is on showing and understanding the discursive practices and motivation of people who deliberately reject parenting. It also concerns contemporary terminological difficulties connected with the concept of family.
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