Schwere und mittelschwere Hämophilie unter prophylaktischer Substitutions therapie

Summary

Due to its influence on haemophilic arthropathy, the evaluation of knee extensor (K_Ext) and flexor (K_Flex) torques plays an important role in the preventive and rehabilitative context of haemophilia. Thus, the present study aimed at investigating maximal static torque (M_Max) of K_Ext and K_Flex. 14 boys with haemophilia (8 severe, 6 moderate; age: 11.7 ± 2.8 years; prophylactic treatment > 5 years) and 14 healthy carefully pair-matched controls (age: 11.5 ± 2.7 years) were separately measured for the left and right leg for M_Max. Furthermore, the ratio K_Flex/K_Ext was calculated and the joint situation assessed using the Haemophilia Joint Health Score. Results: No significant group-effect was observed for M_Max of the K_Ext and K_Flex as well as for the ratio K_Flex/K_Ext (p > 0.05). Despite significant higher joint scores in haemophilic children compared to their healthy controls (p < 0.01), patients merely showed minor joint impairments. Conclusion: Children and adolescents with severe and moderate haemophilia under prophylactic replacement treatment with a good joint status showed comparable maximal strength performance of relevant knee muscles compared to their healthy peers.

Zusammenfassung

Aufgrund der Bedeutung für die Pathogenese der hämophilen Arthropathie, besitzt die Diagnostik der Knieextensoren (K_Ext) und Knieflexoren (K_Flex) einen zentralen Stellenwert im Kontext präventiver und rehabilitativer Fragen hämophiler Patienten. Ziel der vorliegenden Studie war die Untersuchung der maximalen Drehmomente (M_Max) der K_Ext und K_Flex unter statischen Testbedingungen. Die M_Max der K_Ext und K_Flex wurde von 14 Jungen mit Hämophilie (8 schwer, 6 mittelschwer; Alter: 11,7 ± 2,8 Jahre; Prophylaxe > 5 Jahre) und 14 parallelisierte Kontrollprobanden (Alter: 11,5 ± 2,7 Jahre) für beide Beine getrennt erfasst. Ferner wurde das Verhältnis K_Flex/K_Ext ermittelt und die Gelenksituation anhand des Haemophilia Joint Health Scores beurteilt. Ergebnis: Weder für M_Max der K_Ext und K_Flex noch für das Verhältnis K_Flex/K_Ext konnte ein signifikanter Gruppeneffekt nachgewiesen werden (p >0,05). Die Patienten zeigten nur geringe Gelenkauffälligkeiten, unterschieden sich aber von den Kontrollpersonen (p < 0,01). Schlussfolgerung: Kinder und Jugendliche mit Hämophilie haben bei gutem Gelenkstatus unter prophylaktischer Substitutionstherapie eine vergleichbare Muskelkraft der Kniegelenksmuskulatur wie gesunde Gleichaltrige.

Keywords

Haemophilia - children - strength - quadriceps - hamstring muscle

Schlüsselwörter

Hämophilie - Kinder - Kraft - Quadrizeps - ischiokruale Muskulatur

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