A 17-year-old female presented to the emergency department of Deir ez-Zor hospital with loss of consciousness after 3 days of general asthenia, polyuria, anadipsia and vomiting.
- Initial workup in Deir ez-Zor Hospital:
- Emergency laboratory tests revealed abnormalities as shown in (table 1).
Table 1: abnormal findings in Deir ez-Zor hospital |
Glucose
|
600 mg/dl
|
Normal range: 70 – 115 mg/dl
|
Ketone bodies
|
+++
|
Normal range: Negative
|
Creatinine
|
2.9 mg/dl
|
Normal range: 0.5 – 1.3 mg/dl
|
CRP
|
21.9 mg/dl
|
Normal range up to 5 mg/dl
|
Hemoglobin
|
8.4 g/dl
|
Normal range: 11.5 – 16.5 g/dl
|
White blood cells
|
12100 Cells/µl
|
Normal range: 4000 – 10000 Cells/µl
|
2 - Based on the findings, diabetic ketoacidosis (DKA) was diagnosed.
1 - Intravenous (IV) fluids and Insulin therapy were initiated.
2 - Blood sugar levels Improved, but the patient's level of consciousness didn't.
According to the situation, the patient was transferred to Yusuf al-Azma Hospital two days later for further management.
- Physical examination and vital signs upon arrival at Yusuf al-Azma Hospital:
1- Physical examination:
Upon arrival to our hospital, she was unconscious and unresponsive with abdominal distention, a mild erythematous rash on the cheeks and nose, oral ulcers, swollen knees, pitting edema in the lower extremities, and diminished bilateral breath sounds on chest auscultation.
2- Vital signs were as follow:
- Blood pressure (BP): 110/60 mmHg.
- Heart rate (HR): 100 beats per minute (bpm).
- Temperature (Δ): 37 ̊C (axillary route).
- Respiratory rate (RR): 30 breaths per minute.
- The parents reported photosensitivity as the only other significant medical history.
1- Laboratory tests upon arrival showed the following abnormalities (table 2):
Table 2: abnormal findings in Yusuf al-Azma Hospital upon arrival
|
PH
|
6.846
|
Normal range: 7.35 – 7.45
|
pO2
|
56 mmHg
|
Normal range: 75 – 100 mmHg
|
pCO2
|
15.4 mmHg
|
Normal range: 35 – 45 mmHg
|
HCO3
|
3.4 mmol/l
|
Normal range: 22 – 26 mmol/l
|
Hemoglobin
|
8.4 g/dl
|
Normal range: 11.5 – 16.5 g/dl
|
Lympho count
|
600 Cells/µl
|
Normal range: 1500 – 3500 Cells/µl
|
Glucose
|
166.9 mg/dl
|
Normal range: 70 – 115 mg/dl
|
K
|
3.03 mmol/l
|
Normal range: 3.6 – 5.2 mmol/l
|
Creatinine
|
4.3 mg/dl
|
Normal range: 0.5 – 1.3 mg/dl
|
Urea
|
165.27 mg/dl
|
Normal range: 10 – 45 mg/dl
|
Protein in 24h urine
|
0.577 g
|
Normal range: 0 – 0.15 g
|
ESR
|
80 mm/h
|
Normal range: up to 15 mm/h
|
CRP
|
16 mg/dl
|
Normal range: up to 5 mg/dl
|
Antinuclear antibody (ANA)
|
1:160
|
Normal range: Negative (less or equal to 1:40)
|
2- Computed Tomography (CT) of the chest revealed pleural effusion (Figure 1).
- Initial management upon arrival:
1- The patient was unconscious so we put her on a ventilator.
2- Based on the findings in (table 2); Intravenous (IV) fluids (1 liter of saline solution every 6 hours at 125 ml/hr containing 40 ml of KCl), sodium bicarbonate (NaHCO3) (20 ml/hr), and Insulin therapy were initiated to manage the diabetic ketoacidosis (DKA).
3- Multidisciplinary consultation was performed for the patient, with regular assessment to adjust the treatment plan as needed.
- Multidisciplinary consultation result:
1- Renal consultation result: due to the diabetic ketoacidosis, high number of Creatinine and urea in the blood (table 2) they recommended performing daily hemodialysis sessions for the patient.
2- Rheumatology consultation result: according to the physical examination and laboratory tests the patient was diagnosed with juvenile systemic lupus erythematosus ((j)SLE) as she achieved 8 out of 11 of the 1997 American College of Rheumatology (ACR) criteria for classification of SLE (table 3); and they add Hydrocortisone 100mg 3 times daily and Hydroxychloroquine 200mg once at night to the treatment plan:
Table 3: 1997 ACR criteria for the classification of SLE
|
Criteria
|
Findings in the patient
|
1- Malar (butterfly) rash. |
Mild erythematous rash on the cheeks and nose.
|
2- Discoid rash. |
|
3- Photosensitivity |
The parents reported photosensitivity in her medical history.
|
4- Oral ulcers |
Physical examination revealed oral ulcers.
|
5- Arthritis |
Swollen knees.
|
6- Serositis |
Pleural effusion on the chest computed tomography (CT).
|
7- Renal disorder |
Protein in 24h urine = 0.577 g
|
8- Neurological disorders |
|
9- Hematological disorders |
Laboratory tests showed lymphopenia
|
10- Immunological disorders |
|
11- Antinuclear antibody (ANA)
|
Laboratory tests showed ANA positivity.
|
3- Endocrinology consultation result: monitor blood sugar every 6 hours to adjust insulin dose as needed.
4- The cardiology and neurology consultation results were unremarkable.
- Initial management follow-up:
The patient regained consciousness two days after hospital admission, with improvement in blood gas and glucose values, and was successfully weaned off the ventilator. However, the lymphocyte count continued to decrease; interpreted in the context of juvenile systemic lupus erythematosus, and there was slight improvement in renal function test numbers despite daily hemodialysis sessions.
Table 4: patient follow-up after initial management
|
|
Before
|
After
|
Notes
|
PH
|
6.846
|
7.378
|
Normal range: 7.35 – 7.45
|
pO2
|
56 mmHg
|
75 mmHg
|
Normal range: 75 – 100 mmHg
|
pCO2
|
15.4 mmHg
|
18.6 mmHg
|
Normal range: 35 – 45 mmHg
|
HCO3
|
3.4 mmol/l
|
10.7 mmol/l
|
Normal range: 22 – 26 mmol/l
|
Lympho count
|
600 Cells/µl
|
200 Cells/µl
|
Normal range: 1500 – 3500 Cells/µl
|
Glucose
|
166.9 mg/dl
|
119.4 mg/dl
|
Normal range: 70 – 115 mg/dl
|
K
|
3.03 mmol/l
|
3.63 mmol/l
|
Normal range: 3.6 – 5.2 mmol/l
|
Creatinine
|
4.3 mg/dl
|
3.48 mg/dl
|
Normal range: 0.5 – 1.3 mg/dl
|
Urea
|
165.27 mg/dl
|
163.02 mg/dl
|
Normal range: 17 – 43 mg/dl
|
- On the fifth day, the patient developed right ptosis, blurred vision in the right eye, mydriasis with no light reflex, a feeling of heaviness followed by loss of movement in the right eye in all directions, during the patient's assessment, a painless white lesion was found on the roof of the palate with thick brown secretions and crusts in the nasal cavity, and a biopsy was taken from it. This was followed by respiratory failure and loss of consciousness, so the patient was re-intubated and connected to a ventilator. The multidisciplinary team suspected cavernous sinus thrombosis in the context of a lupus flare, and Magnetic Resonance Imaging (MRI) with contrast was requested. Unfortunately, MRI is not available in our hospital or any surrounding facilities, additionally, the patient has kidney failure; so a Computed tomography (CT) of the brain was performed, which only showed mucosal thickening of the maxillary and ethmoid sinuses (Figure 2).
- On the sixth day, the biopsy showed that the patient had Aspergillus and Mucormycosis infection. There was bony exposure on the midline of the palatal vault and severe necrosis around it (Figure 3).
- Due to the kidney failure, Amphotericin was given at a reduced dose of 50mg IV every 24h after hemodialysis sessions. The surgical team decided to perform a palatal vault resection with ethmoid curettage, but unfortunately, the patient passed away before any intervention could be performed.