Рідкісні діагнози для нашої лікарні не є викликом. #Клініка_захворювань_та_реабілітації_дітей_раннього_віку береться за встановлення найскладніших діагнозів у дітей, залучаючи до цього усіх потрібних фахівців медичної справи. І нещодавний випадок цьому підтвердження.
захворювання або синдроми, в основі, яких лежать порушення обміну ферментних систем в організмі, що приводять до ушкодження канальцевої секреції або реабсорбції.
Рідкісне генетичне захворювання, діагностувати яке досить важко.
Все почалося з того, що у приймальне відділення нашої лікарні звернулися батьки із півторамісячним хлопчиком Максимчиком.
Родина родом із Харкова, змушена втікати від війни в одну із відносно спокійніших областей Західної України, де і запідозрили вроджену аномалію розвитку нирок.
До нашої лікарні Максимко був скерований для повного урологічного обстеження, але при огляді дитини у приймальному відділенні, клінічно виступали на перший план ознаки різкого зневоднення, м’язевої гіпотонії, виражені ознаки інтоксикації, млявість, апатія, дефіцит маси тіла. Оскільки ми зробили висновок, що у дитини є ознаки білково-енергетичної недостатності, то вона поступила одразу до реанімаційного відділення для нормалізації стану.
Коли було проведено ряд перших обстежень, то виявилося, що у дитини виражений метаболічний ацидоз. Тобто, порушена кислотно-лужна рівновага організму і електролітні розлади – зменшений рівень калію в крові, підвищений рівень кальцію.
«І фактично ця порушена рівновага наштовхнула нас на клінічний і діагностичний пошук ймовірного захворювання. Бо у дитини були стійкі зміни в аналізі сечі: поліурія, протеїнурія, лейкоцитурія, кальціємія і гіпокаліємія, плюс переважаючі ознаки метаболічного ацидозу. Все це в комплексі наштовхнуло нас на думку, що можливо у дитини одна із видів тубулопатії, яка зустрічається доволі рідко і важко діагностується у дітей», – прокоментувала керівниця Клініки Оксана Короляк.
Діагностичний пошук лікарів сягнув далі – медики почали забір аналізів на специфічні обстеження, моніторити електролітний обмін, гази крові, що в комплексі підтвердило рідкісне і важке захворювання. Дитина була додатково проконсультована ендокринологом, генетиком та головним нефрологом України, професором Дмитром Івановим, а скринінгові обстеження надіслані до однієї із клінік США.
Весь цей час дитина отримувала терапію, коригувався її електролітний обмін, коригувалися гази крові, лужна рівновага тощо і на цьому тлі дитині ставало краще.
У Максимка збільшилася вага, зникли прояви інтоксикації, він ставав активніший, у нього нормалізувалися усі електролітні розлади, санувалася сеча, йому було проведено ще ряд обстежень та заходів для покращення стану здоров’я і через півтори місяці перебування у Клініці хлопчик у задовільному стані був виписаний додому.
За кілька днів після виписки прийшло і підтвердження діагнозу із США – тубулопатія: нирковий тубулярний ацидоз II типу. Сьогодні Максимко під постійним спостереженням нефролога, ендокринолога та генетика, відвідує Катамнестичний центр «Малюк» нашої лікарні.
«Максимчик має шанс на повноцінне життя, приймаючи коригуючу терапію. Специфічного лікування тут немає, адже у його нирках внаслідок метаболічних розладів утворюються камінчики – нефрокальцинати, які не дають повноцінно працювати нирці. Це генетичне захворювання, яке в тому числі може бути пов’язане із недостатністю паращитовидних залоз. Тобто, дослідження тривають», – розповідає лікарка.
Ми бажаємо нашому Максимку подолати цю недугу, батькам витримки та сил пройти цей не простий шлях разом!
Звертайтеся до нашої дитячої лікарні:
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