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    Adult-Onset Immunodeficiency Mainly Characterized by the Clinical Manifestations of Systemic Polyserositis and Infection
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    Abstract:
    Keywords:
    Hongmei Din, Hongbin Fan, Deqing Geng
     
    Hospital of Xuzhou Medical College, Xuzhou, 221003, People’s Republic of China
     
    Abstract: According to the report of The New England Journal of Medicine, under the joint research of medical researchers from Taiwan, Thailand and the United States and other areas, it was confirmed that there was a new disease in the world, named ‘Adult-Onset Immunodeficiency’. The media called it as ‘AIDS-like disease’ due to AIDS-like symptoms of low immunity (lesser CD4+ T cells). However,  different form AIDS, the HIV virus unable to detected from the AIDS-like disease. According to media reports, the world has so far been diagnosed with AIDS-like disease more than 200 cases[1].
     
    Keywords: AIDS-like disease;more serous cavity involvement;hydrops[H1] ;infection;case report
     
    Citation: Din H et al, Adult-Onset Immunodeficiency Mainly Characterized by the Clinical Manifestations of Systemic Polyserositis and Infection, J Gen Neuro 2016; 1(1):
     
     
    *Correspondence to:
     
    Received:                          ; Accepted:                        ; Published Online: TBA
     
     
    AIDS-like disease patients exhibit common AIDS-like symptoms of low immunity (lesser CD4+ T cells). But different form AIDS, the HIV virus can’t be detected from the AIDS-like disease under current detection techniques. Recently, asians were the major part of the group that has been diagnosed with AIDS-like disease, mostly from Thailand and Taiwan. The average age of onset was about 50 years old. The studies showed that, tropical and subtropical regions like the Southeast Asia and Taiwan, due to the hot humid climate and dense population results in high incidence of AIDS-like disease. AIDS-like disease caused patient’s body to produce hormone specific antibody (anti–interferon-γ autoantibodies) that can attack and engulf the white blood cell, leading to a decline in the body’s immune system against mold, mycobacterium, salmonella and other specific bacterial infections. The patients showed AIDS-like clinical symptoms, such as recurrent fever, bone and skin fungal infections, and even systemic mixed infection. Characteristics of the disease of being contagious and the treatment are still unknown.
     
    Case data
     
    Personal data
    Male and 24 year’s old. Single. Work location before infected disease was in Suzhou, China. No alcohol consumption and smoking.
     
    Medical records
    Recurrent fever for 2 months and unconsciousness for 15 days. Admitted to the hospital on May 14th, 2012 (Admission Number: 0619882). 
     
    Medical and family history
    The patient had 15 years of hepatitis B, and physical health. No family history of related diseases.
     
    Physical examination
    Temperature: 37.5℃, pulse: 95 beats per minute, respiratory rate: 18 breaths per minute, blood pressure: 110/70 mmHg. The skin has no obvious bleeding and jaundice, and the lymph nodes were not swollen. Both lungs have respiratory harshness. Moist rales were heard during both the inspiration and expiration. The rhythm of the heart was normal with no pathological murmur. The abdomen was soft, the liver and spleen were not swollen under the ribs. No masses were swollen on the both lower extremities.

    Physical examination of neurological system
    He has some shallow coma, equal size of the pupil in both eyes with the diameter approximately 2.5mm, and positive for pupillary light reflex. Both side of nasolabial sulcus was symmetrical, the muscular tension is low to be able to voluntary movement. Pain stimulation can make the limbs activity, double upper limb tendon hyperreflexia, double lower limbs normal tendon reflex, double lower limbs pathological positive. The other symptoms like neck resistance and positive brinell character and gram were detected. The patients refused to be examined for other examination.
     
    Examination prior to admission
    There was nucleated cell (2*10*6/l), chloride tendency (138.7mmol/l), sugar tendency (4.1mmol/l) and protein (1.036g/l) detected in the cerebrospinal fluid, and the TB-DNA was negative. Bacteria, acid bacillus and fungus were not detected (sample was outsource to the 3rd party on May 2nd, 2012).
     
    Viral examination
    The Hepatitis B surface antigen, HBeAb, and HbcAb were positive, the syphilis antibody and antibodies to HIV were negative (sample was outsource to the 3rd party on May 2nd, 2012); HBV-DNA(1.0×10*3copies/ml) (sample was outsource to the 3rd party on May 3rd, 2012); Hydrothorax  displayed light yellow and slightly mix. Li Fan check is positive, with karyocyte (2352×10*6/l), total protein (35.9g/l), Carbohydrate (7.5mmol/l), ADA (11U/L), LDH(352U/L) and CPR(33.39mg/l), bacteria and acid bacillus were not detected (sample was outsource to the 3rd party on May 4th, 2012). No bacterial growth in blood culture (sample was outsource to the 3rd party on May 7th, 2012).
     
    Biochemistry test: Albumin( 28.4g/l),cereal third transaminase (42U/L),aspertate aminotransferase (5942U/L) (Sample was outsource to the 3rd party on May 9th, 2012).
     
    Blood examination
    White blood cells (16.34×10*9/1), neutrophils (9.89×10*9/l), eosinophils (3.04×10*9/l), erythrocyte (3.23×10*12/l), hemoglobin (96g/l) (sample was outsource to the 3rd party on May 9th, 2012).
     
    Electrolyte
    Potassium (3.98 mmol/l), sodium (132.5 mmol/l), chloride (93.2 mmol/l), no abnormal in urine routine, tumor marker, brain natriuretic, cortisol, ACTH, FSH, LH, E2 and thyroid function (sample was outsource to the 3rd party on May 12th, 2012).
     
    Chest CT
    Under the two lungs it was infected with tuberculosis, effusion in the two side pleural, inner mediastinal lymphadenopathy, and increase on both sides of the axillary were detected (sample was outsource to the 3rd party on May 2nd, 2012).
     
    Skulls CT
    No obvious abnormal (sample was outsource to the 3rd party on May 2nd, 2012); [Epigastrium color Doppler ultrasound] Liver area photoelectric was intensive, no abnormal in the gallbladder and pancreas, the spleen enlargement and the effusion in the right pleural were detected (sample was outsource to the 3rd party on May 4th, 2012).
     
    Double kidneys colour Doppler ultrasound
    No obvious abnormal (sample was outsource to the 3rd party on May 9th, 2012).
     
    MRI enhancement scanning
    No obvious abnormal (sample was outsource to the 3rd party on May 10th, 2012).
     
    Encephlogram
    Had a light to moderate abnormal, the delta and the theta frequency band energy  was increased (sample was outsource to the 3rd party on May 13th, 2012).
     
    Instant examination after admission
    According to the blood examination: white blood cell (11.86×10*9/l), neutrophils (10.70×10*9/l), lymphocyte(0.5×10*9/l), RBC(3.79×10*12/l), hemoglobin(105g/l); According to the blood biochemical examination: Albumin (31.7 g/l), cereal third transaminase( 61 u/l), aspertate aminotransferase (199 u/l), and no abnormal in renal function, blood fat and blood electrolytes.
     
    The patient was in sudden unconsciousness on next day 8 am. The physical examination revealed that the pupil size of both eyes was not equal, the diameter of the left side was 4.0mm, 6.0mm in diameter on the right side, and papillary reaction to light was absent. The possibility for cranial cerebral hernia caused by high pressure formation was considered. The patient was given mannitol dehydration of intracraninal pressure, after 10 minutes the patients return to consciousness. The pupil size of both eyes turn equal, the diameter was about 3.0mm, and exist the pupillary reaction to light. Next morning, he was given Lumbar puncture cranial pressure measurement (200mmH2O) and examination of cerebrospinal fluid, it revealed that Pan’s experiment (2+,WBC-BF9.0×10*6/l), single nuclear cell number (6.0×10*6/l); Biochemistry (GLU4.1mmol/l,Cl117.3mmol/l, PROT3.00g/l). He was given treatment for chemotherapy, dehydration of intracraninal pressure to fight infection, protect gastric mucosa and supporting control treatment. The patient had diarrhea on May 18th, the stool examination revealed that the sample was yellow and pus, Microscopy 3 + WBC, RBC microscopy 2 +, fat ball and parasites were not detected. The patients diarrhea stopped after treatment. Granulocyte obvious hyperplasia and enlargement of particles were detected through the bone marrow examination given on May 24th. The amount of pleural effusion and a small amount of pleural effusion were respectively detected on the left side and the right side. The patient was given pleurocentesis immediately, and the hydrothorax displayed light yellow and slightly mix. Li Fan check is positive, WBC-BF (430.0×10*6/l), single nuclear cell number (284.0×10*6/l); Biochemistry examination revealed TP34.9g/l, ALB19.8g/l, LDH520U/L, ADA18U/L. After repeated chest colour to exceed examination, the bilateral pleural effusion was gradually reduced after times of puncture treatment. The immunofluorescence blood tests revealed the result (CD375%, CD423%, CD849%, CD4/CD80.47, CD191%CD16+CD5619%). Hospitalization for autoimmune series are negative, ANCA,HIV and TP were negative, ASO and ESR were normal, hs-c-reactive protein(22.80mg/L). A small amount of effusion on the right side of bowel loops was detected in the abdominal ultrasonography on June 5th. Little amount of pericardial effusion was detected in the UGG on June 7th. Cranium CT, MRI scan + enhance and Cranium CTV were normal. The pancreas volume swell, and the dim in peripancreas, retroperitoneal, and the fat around the local loops space, pleural effusion with lower lobe expansion both lungs, the pericardium a small amount of effusion were detected in the upper abdominal CT scan on May 21st. The result of blood amylase check was normal. The chest CT on May 24th revealed that bilateral pleural effusion and the two lungs atelectasis; Within the mediastinum and two axillary slightly enlarged lymph nodes in shadow; Center of the pelvic scan + epigastric enhancement on June 8th revealed: 1. belly bowel mild expansion effusion, pelvic a small amount of effusion, little older retroperitoneal lymph nodes; 2. bilateral pleural effusion and the two lungs atelectasis; a small amount of effusion was detected in the pericardium. Colour Doppler ultrasound on June 26th revealed there was a small amount of pleural effusion on both sides. The patients had increasing fever up to 40.0℃, he was given Lai ammonia aspirin or indomethacin suppository processing fever, but without obvious antipyretic effects. With encephalitis and pleuritis gradually improving, fever was also gradually decreased. The left upper lobe patch article showed in the right pleural thickening slightly between left was detected in the Check CT On July 3rd. The fever and effusion were improved after treatment and left hospital on July 14rd. He had occasional fever when he went back to his hometown Anhui, and back to normal body temperature after taking fever-reducing medication. He had a fever again three days after returning to Suzhou in August, with rising temperature, then was admitted by the Beijing 301 hospital. Latent tuberculosis infection was considered and gave him anti-tuberculous therapy, with effection. The patient was readmitted to our hospital in September and exist pleural effusion, so gave him chemotherapy and anti-infection treatment for more than 20 days, the fever was improved, but had a fever again after a week. Patient had a pain around the right knee when he was admitted second time, the knee joint ray films was performed, without abnormal.
     
     
    Discussion
     
    Adult-Onset Immunodeficiency was first discovered in 2004 by Sarah Brown (the national institute of allergy and infectious diseases of USA, female scientist) is currently in conjunction with Thailand and Taiwan scientists for research. Since she began to study, in a short span of six months she collected 100 cases, a total of 200 cases were reported so far. Since the collected cases were from Asians, the scholars from Taiwan who was involved in the research call it the ‘Asian new immunodeficiency syndrome’[1].According to the report of The New England Journal of Medicine published on August 23rd, over the past eight years, most cases were found in Thailand and Taiwan, including some Asians in the United States[1]. Brown said, this was another kind of acquired immune deficiency syndrome, the average age of onset was about 50 years old[4]. This is a kind of adult immunity caused by some causes serious decline, susceptible to mold mycobacterium certain bacteria such as salmonella infection[2]. These patients were reported just like the Vietnamese, americans RuanJin, reported by Brown, had long-term fever again and again and even systemic mixed infection. It caused the condition to repeat, just like having pulmonary tuberculosis. But the mycobacterium tuberculosis and HIV was negative after repeat check. The case we reported were in early onset, there was a pain on the right side of the knee joint. Whether it is early bone infection, it remains to be fastidious.
     
    Postscript Note: The patient had a high fever and respiratory airway infection after half a year, and was admitted to our hospital. Unfortunately, the patient able to survived for a month because of systemic multiple organ failure.
     
    References
    1.      Browne SK, Burbelo PD, Chetchotisakd P, Suputtamongkol Y, Kiertiburanakul S, et al. Adult-Onset Immunodeficiency in Thailand and Taiwan. N Engl J Med 2012; 367(8): 725–734.
    2.      Marchione M. New immune-system disease found in Asians; causes AIDS-like symptoms in people without HIV [Internet]. The Washington Post. [cited 2012 August 22).
    3.      New AIDS-like mystery disease. The Voice of Russia. [Cited 2012 August 22].
    4.      AIDS 2.0: Highly contagious disease spreading in China. The Swash. [Cited 2012 August 22]. 
    5.      Kent Sepkowitz. New Thai-Taiwanese Syndrome Is Not AIDS 2.0. The Daily Beast. [Cited 2012 August 22].
    6.     Adult-onset immunodeficiency syndrome [Internet]. Wikipedia. [Cited 2012 September 15].

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