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1.
The Korean Journal of Critical Care Medicine ; : 140-145, 2016.
Artículo en Inglés | WPRIM | ID: wpr-770933

RESUMEN

Severe hyperammonemia can occur as a result of inherited or acquired liver enzyme defects in the urea cycle, among which ornithine transcarbamylase deficiency (OTCD) is the most common form. We report a very rare case of a 45-year-old Korean male who was admitted to the intensive care unit (ICU) due to severe septic shock with acute respiratory failure caused by Pneumocystis jiroveci pneumonia. During his ICU stay with ventilator care, the patient suffered from marked hyperammonemia (>1,700 µg/dL) with abrupt mental change leading to life-threatening cerebral edema. Despite every effort including continuous renal replacement therapy and use of a molecular adsorbent recirculating system (extracorporeal liver support-albumin dialysis) to lower his serum ammonia level, the patient was not recovered. The lethal hyperammonemia in the patient was later proven to be a manifestation of acquired liver enzyme defect known as OTCD, which is triggered by serious catabolic conditions, such as severe septic shock with acute respiratory failure.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Amoníaco , Edema Encefálico , Hiperamonemia , Unidades de Cuidados Intensivos , Hígado , Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa , Ornitina Carbamoiltransferasa , Ornitina , Pneumocystis carinii , Neumonía , Terapia de Reemplazo Renal , Insuficiencia Respiratoria , Choque Séptico , Urea , Ventiladores Mecánicos
2.
Korean Journal of Critical Care Medicine ; : 140-145, 2016.
Artículo en Inglés | WPRIM | ID: wpr-42557

RESUMEN

Severe hyperammonemia can occur as a result of inherited or acquired liver enzyme defects in the urea cycle, among which ornithine transcarbamylase deficiency (OTCD) is the most common form. We report a very rare case of a 45-year-old Korean male who was admitted to the intensive care unit (ICU) due to severe septic shock with acute respiratory failure caused by Pneumocystis jiroveci pneumonia. During his ICU stay with ventilator care, the patient suffered from marked hyperammonemia (>1,700 µg/dL) with abrupt mental change leading to life-threatening cerebral edema. Despite every effort including continuous renal replacement therapy and use of a molecular adsorbent recirculating system (extracorporeal liver support-albumin dialysis) to lower his serum ammonia level, the patient was not recovered. The lethal hyperammonemia in the patient was later proven to be a manifestation of acquired liver enzyme defect known as OTCD, which is triggered by serious catabolic conditions, such as severe septic shock with acute respiratory failure.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Amoníaco , Edema Encefálico , Hiperamonemia , Unidades de Cuidados Intensivos , Hígado , Enfermedad por Deficiencia de Ornitina Carbamoiltransferasa , Ornitina Carbamoiltransferasa , Ornitina , Pneumocystis carinii , Neumonía , Terapia de Reemplazo Renal , Insuficiencia Respiratoria , Choque Séptico , Urea , Ventiladores Mecánicos
3.
Korean Circulation Journal ; : 504-507, 2013.
Artículo en Inglés | WPRIM | ID: wpr-167931

RESUMEN

A 65 year-old female with a history of xerostomia and xerophthalmia was presented with dyspnea on exertion (New York Heart Association class III). Echocardiography and cardiac catheterization demonstrated severe pulmonary hypertension (PH). Laboratory examinations showed positive anti-nuclear and anti-Ro/SS-A antibodies. Schirmer's test was positive and salivary gland scintigraphy revealed severely decreased tracer uptakes in both parotid and submandibular glands. By excluding other possible causes of PH during further examinations, she was diagnosed with severe PH associated with primary Sjogren's syndrome. Her dyspnea symptom was much improved with endothelin receptor antagonist and azathioprine.


Asunto(s)
Femenino , Humanos , Anticuerpos , Azatioprina , Cateterismo Cardíaco , Catéteres Cardíacos , Disnea , Ecocardiografía , Corazón , Concentración de Iones de Hidrógeno , Hipertensión Pulmonar , Receptores de Endotelina , Glándulas Salivales , Síndrome de Sjögren , Glándula Submandibular , Xeroftalmia , Xerostomía
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