Sirpjveida šūnu anēmija ar krīzi: klīniskā gadījuma apraksts.
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Date
2020
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Rīgas Stradiņa universitāte
Rīga Stradiņš University
Rīga Stradiņš University
Abstract
Atslēgas vārdi. Sirpjveida šūnu anēmija ar krīzi, hemolītiska anēmija, vazookluzīvs sāpju sindroms, akūts krūšu sindroms
Ievads. Sirpjveida šūnu anēmija (SŠA) ir iedzimta autosomāli recesīva slimība, kurai raksturīga hroniska hemolītiska anēmija ar akūtām sirpjveida šūnu anēmijas krīzes epizodēm, notiekot asinsvadu oklūzijām un plaušu infarktiem.
Akūts krūšu sindroms ir biežs akūtu plaušu slimību izraisītājs bērniem ar SŠA. Slimība novērojama 1 no 600 afroamerikāņu jaundzimušajiem. Puse no SŠA pacientiem dzīvo Subsahāras Āfrikā, Rietumāfrikā un Indijā.
Klīniskā gadījuma apraksts. Sešpadsmit gadu vecs Nigēriešu izcelsmes zēns tika nogādāts reģionālajā slimnīcā pēc piecu dienu ilgām, pieaugošām muguras un kāju sāpēm. Pacientam bija vemšanas epizodes un ķermeņa temperatūra sasniedza 39°C. Slimnīcā pacients saņēma antibiotikas, hidratācijas terapiju un NSPL sāpju atvieglošanai. Pēc četrām dienām slimnīcā pacienta stāvokļa uzlabošanās netika novērota: ķermeņa temperatūra saglabājās 39°C, anēmija bija progresējusi un kopējais bilirubīna līmenis bija pieaudzis.
Pacients tika pārvests uz Bērnu Klīnisko universitātes slimnīcu, kur saņēma eritrocītu masas transfūziju, jo hemoglobīna līmenis bija nokrities līdz 5 g/dL. Papildus tam, pacients saņēma fentanilu un NSPL sāpju atvieglošanai, kā arī i/v hidratāciju. Pacientam tika novērota sklēru un gļotādas dzelte. Asins analīze uzrādīja palielinātu leikocītu skaitu, kā arī CRO, IL-6 un D-dimēru līmeni. Rentgena uzņēmumi, kas tika veikti skābekļa desaturācijas dēļ, atklāja abpusējus bazālus aizēnojumus. Tika izvirzīta diferenciāldiagnoze starp pneimoniju un akūtu krūšu sindromu. Seroloģiskie izmeklējumi neuzrādīja Chlamydia pneumoniae un Mycoplasma pneumoniae klātbūtni. Pacients tika izrakstīts pēc 4 dienām ar daļējiem veselības stāvokļa uzlabojumiem.
Secinājumi. Sirpjveida šūnu anēmija visbiežāk klīniski izpaužas ar vazookluzīvām sāpju epizodēm mugurā un ekstremitātēs. Slimību iespējams veiksmīgi ierobežot, nodrošinot pietiekamu hidratāciju, NSPL un opioīdus. Gadījumā, kad pacientam novērojamas vazookluzīvas sāpju epizodes, drudzis, desaturācija un jauni plaušu infiltrāti krūškurvja rentgena uzņēmumos, nepieciešams izvērtēt iespējamu akūta krūšu sindroma attīstību.
Kopsavilkums. Sešpadsmit gadus vecs zēns no Nigērijas tika hospitalizēts ar sirpjveida šūnu anēmijas krīzi, kas izpaudās ar hemolīzi un vazookluzīvu sāpju epizodi un aizdomām par iespējamu akūta krūšu sindroma attīstību.
Key words. Sickle cell crisis, haemolytic anaemia, vaso-occlusive pain event, acute chest syndrome Introduction. Sickle cell disease (SCD) is an inherited autosomal recessive disorder characterized by chronic haemolytic anaemia and episodes of acute sickling crisis due to vascular occlusion and infarction. Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with SCD. The incidence at birth is 1 in 600 African American newborns and half of the SCD patients reside in sub-Saharan and Western Africa and India. Case report description. A 16 year old Nigerian boy was admitted to a regional hospital after five days of increasing leg and back pain. The patient was vomiting, with a body temperature of 39°C. At the hospital the patient received antibiotics, hydration and NSAID for pain relief. After 4 days at the hospital there was no improvement in the patient’s condition: temperature remained at 39°C, anaemia had progressed, and the level of total bilirubin had increased. The patient was transferred to the Children’s Clinical University Hospital where he received erythrocyte mass transfusion due to haemoglobin level of 5 g/dL. In addition, he received Fentanyl and NSAID for pain relief and I/v hydration. Jaundice of the sclerae and mucous membranes was observed. Blood analysis showed increased leukocytes, CRP, IL-6 and D-dimer. Due to desaturation chest X-ray was performed revealing bilateral basal opacities posing the differential diagnosis of pneumonia and acute chest syndrome. Serologic testing for Chlamydia pneumoniae and Mycoplasma pneumoniae was negative. After 4 days the patient was discharged with partial improvements in his condition. Conclusion. Vaso-occlusive pain event in the back and extremities is the most common clinical manifestation in patients of SCD. It is well managed with an adequate hydration, NSAID and opioids. Evolvement of ACS should be considered in patients presenting with vaso-occlusive pain event, fever, desaturation and new pulmonary infiltrate on chest X-ray. Summary. A 16-year old Nigerian boy with sickle cell crisis presented with haemolysis and vaso-occlusive pain event with suspicion of possible evolvement of acute chest syndrome.
Key words. Sickle cell crisis, haemolytic anaemia, vaso-occlusive pain event, acute chest syndrome Introduction. Sickle cell disease (SCD) is an inherited autosomal recessive disorder characterized by chronic haemolytic anaemia and episodes of acute sickling crisis due to vascular occlusion and infarction. Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with SCD. The incidence at birth is 1 in 600 African American newborns and half of the SCD patients reside in sub-Saharan and Western Africa and India. Case report description. A 16 year old Nigerian boy was admitted to a regional hospital after five days of increasing leg and back pain. The patient was vomiting, with a body temperature of 39°C. At the hospital the patient received antibiotics, hydration and NSAID for pain relief. After 4 days at the hospital there was no improvement in the patient’s condition: temperature remained at 39°C, anaemia had progressed, and the level of total bilirubin had increased. The patient was transferred to the Children’s Clinical University Hospital where he received erythrocyte mass transfusion due to haemoglobin level of 5 g/dL. In addition, he received Fentanyl and NSAID for pain relief and I/v hydration. Jaundice of the sclerae and mucous membranes was observed. Blood analysis showed increased leukocytes, CRP, IL-6 and D-dimer. Due to desaturation chest X-ray was performed revealing bilateral basal opacities posing the differential diagnosis of pneumonia and acute chest syndrome. Serologic testing for Chlamydia pneumoniae and Mycoplasma pneumoniae was negative. After 4 days the patient was discharged with partial improvements in his condition. Conclusion. Vaso-occlusive pain event in the back and extremities is the most common clinical manifestation in patients of SCD. It is well managed with an adequate hydration, NSAID and opioids. Evolvement of ACS should be considered in patients presenting with vaso-occlusive pain event, fever, desaturation and new pulmonary infiltrate on chest X-ray. Summary. A 16-year old Nigerian boy with sickle cell crisis presented with haemolysis and vaso-occlusive pain event with suspicion of possible evolvement of acute chest syndrome.
Description
Medicīna
Medicine
Veselības aprūpe
Health Care
Medicine
Veselības aprūpe
Health Care
Keywords
Sirpjveida šūnu anēmija ar krīzi, hemolītiska anēmija, vazookluzīvs sāpju sindroms, akūts krūšu sindroms, Sickle cell crisis, haemolytic anaemia, vaso-occlusive pain event, acute chest syndrome