**8. Urogenital system in immunocompromised**

186 Immunodeficiency

patients are as detailed below;

*Candida albicans*, Coccidioides spp Parasitic - toxoplasmosis, strongyloides.

and occasionally, peripheral nerve injury.

cranial nerve palsies can also occur but are not as common.

**7. Musculoskeletal system in immunocompromised** 

CNS infections caused by infective agents are rare in immunocompetent host, but more frequent in immunocompromised patients. The spectrum of causative organisms may vary greatly, depending on the underlying malignancy, its treatment and various other factors. Infections that had been reported to cause neurological diseases in immunocompromised

Bacterial – staphylococcus spp, pneumococcus, haemophilus spp, mycobacterium tuberculosis, mycobacterium Avium/ Intracellulare Complex, Listeria spp, norcadia spp. fungal *– Cryptococcus neoformans, Aspergillus fumigatus, Zygomycetes (Mucor and Rhizopus),* 

Development of neurologic manifestations depends on a variety of factors, including therapy with drugs like antiretroviral drugs and the patient's overall degree of immunosuppression. Heavily immunocompromised patients like those after allogeneic stem cell transplantation (SCT) or previous T cell depleting treatment regimens (e.g. with fludarabine or alemtuzumab) are at highest risk for cerebral infections. . The infections can cause global or focal cerebral dysfunction, subhemispheric impairment, spinal cord injury

Thus, in the immunosuppressed patient with neurological involvement there are three interrelated areas to consider. First, has whatever caused the immunosuppression either directly or indirectly affected the nervous system? Second, are such problems due to an infection of the nervous system? And last, are there any medical complications that might produce a neurological disturbance? In assessing immunosuppressed patients, the clinician must remember that more than one of these factors may be involved in the neurological presentation. Clinical presentations in these patients may include headache, signs and symptoms of increased intracranial pressure, and lateralizing signs appropriate to the area(s) of involvement. These symptoms can include behavioral, cognitive, and personality changes. Focal symptoms include hemiparesis, aphasia, and visual field defects. Ataxia, seizures, and

Meningoencephalitis is a common presentation however the classical symptoms and signs may be absent in these patients.. Also cerebral toxoplasmosis, cerebral lymphoma and cerebral vascular accident in immunocompromised patients may have similarities in their clinical presentations. The nature of neurological manifestation in patients with impaired immune state also varies with the cause and the degree of the immunosuppression. There is need for high index of suspicion to avoid delay or misdiagnosis that may lead to delay in intervention.

The immune cells and mediators had been implicated in some musculoskeletal diseases. The bone marrow is the source of various haematological cells including the primordial immune

Viral – herpes simplex virus, JC virus, cytomegalovirus, varicella zoster virus.

The urogenital tract contain both cellular and non cellular innate immune components. This ensure the sterility of the urinary tract and part of the genital tract. In immunodeficiency state the urogenital tract are exposed to higher prevalence of both common and rare infections. The urogenital diseases that have been reported in immunocompromised patients include urinary tract infection, epididymitis, prostatitis, extensive condylomata of the urethra, renal abscess and other renal related diseases.

The occurrence of urinary tract infection and its clinical impact is determined, as with any infectious disease, by the interaction between the virulence of the infecting organism and the host defense mechanisms that can be mobilized. In the case of urinary tract infections, an anatomically and functionally intact kidney and urinary tract are the primary host defenses, with phagocytic function and immune mechanisms coming into play to limit the consequences of those infections.

Defects in the immune system determine the clinical manifestations and severity of urinary tract infections (UTI) and the rates of complication. However they only have an indirect role in influencing susceptibility to infection. Of all the categories of immunocompromised hosts, the renal transplant patient is the one most susceptible to the direct and indirect consequences of urinary tract infections. The rates of UTI in diabetics, renal transplant, recipients, neutropenic patients, and patients with AIDS are primarily determined by the degree and duration of urinary tract manipulation, and the higher perineal prevalence of potential pathogens that result from frequent hospitalization and antimicrobial use.

Urogenital tract infection has a different clinicoradiological presentation in immunocompromised patients, with predominance of systemic symptoms, multiple parenchymatous renal foci, and lower frequency of lesions of the collecting system. In the context of immunosuppression, Urogenital tract infection behaves as a severe bacterial infection, with bacteremia and visceral metastatic foci.

Pattern of Clinical Presentations in Immunocompromised Patient 189

immunosupression, the endemic infections, the system or organ with predominant injury, and other associated diseases like Malignancies and infiltrative diseases. It is noteworthy that these patients may have atypical presentations. Thus there is need for surveillance and high index of suspicion of injuries/diseases in these patients to ensure early diagnosis and

Ayaz A, Biviji MD, Guy D. et al. musculoskeletal manifestation of human immunodeficiency

Ana Rañó, Carlos Agustí, Natividad Benito et al. Prognostic Factors of Non-HIV Immunocompromised Patients with Pulmonary Infiltrates. CHEST (2002); 122 (1): 253-

C Dougan, I Ormerod. A neurologist's approach to the immunosuppressed patient. J Neurol

Dawn McGuire. Neurologic Manifestations of HIV .HIV InSite Knowledge Base Chapter

Figueiredo AA, Lucon AM, Júnior RF, Ikejiri DS, Nahas WC, Srougi M. Urogenital tuberculosis in immunocompromised patients. Int Urol Nephrol. 2009;41(2):327-33.

Foroughi et al. Prevalence of dermatologic manifestations among people living with HIV/AIDS in Imam Khomeini Hospital in Tehran, Iran. Journal of AIDS and HIV

Fred A. Lopez, Charles V. Sanders. Recognizing Cutaneous Signs of Infection in

Immune Deficiency Foundation. *Primary Immunodeficiency Diseases.* Family Handbook;

J. Harold Helderman, Simin G. Gastrointestinal complications of transplant

Javier Munoz, Philip Kuriakose. Rash in an Immunocompromised Patient. JAMA.

Kahn LH. The growing number of immunocompromised. Bulletin of the Atomic Scientists

Nancy Lane. Rheumatologic and musculoskeletal manifestation of HIV. HIV inSite

Nick Murphy, Tony Whitehouse, Mark Cook. Immunocompromised; PACT (2010): 1 –57

Neurosurg Psychiatry 2004;75:i43-i49 doi:10.1136/jnnp.2003.035071

Immunocompromised Patients. Hematol Oncol. 2003;6(3): 1 – 4

immunosuppression. J Am Soc Nephrol 13: 277–287, 2002

Elizabeth Boskey Immunocompromised. www/ *About.com (2010)* 

Research Vol. 4(2), pp. 56-59, February 2012

Baxter. Fourth Edition. 2007 : Chapter XI.

intervention.

**Author details** 

**10. References** 

261

June 2003

Epub 2008 Jul 22.

(2012);307(6):612-613. .

(2008): www/thebulletin.org

Knowledge Base chapter 1998.

Umezurike Hughes Okafor

*Enugu State University Teaching Hospital, Parklane, Enugu, Nigeria* 

virus. Am Acad Ortho Surgeons; 10(3): 833-838

Many patients are asymptomatic. Symptoms that may occur include dysuria, urinary frequency and incontinence, flank pain, and fever. Confusion and delirium are often attributed to UTI, although without high fever or sepsis. Uncomplicated UTI is unlikely to cause serious central nervous dysfunction. The clinical signs and follow up of these infections were straight forward in half of the cases. However, in some patients, the infection is fulminant with progression to an abscess despite the use of antibiotics or is unusual because of the pathogens isolated.

Mycobacterial agents causing UTIs are less frequent in immunocompetent individuals; they are more common and severe in immunocompromised individuals. Extra pulmonary tuberculosis (EPTB) represents a progressively greater proportion of new cases and the genitourinary tract is the most common site of EPTB. The most common causative organism of kidney and urinary tract tuberculosis is the Mycobacterium tuberculosis, and occasionally Mycobacterium bovis can also be responsible. Mycobacterium tuberculosis (MTB) has an important impact on kidney transplant recipients, particularly during the first year after surgery. Tuberculosis of the urinary tract is easily overlooked. Symptoms that sometimes occur include back, flank and suprapubic pain, hematuria, frequency, and nocturia. These might also suggest conventional bacterial urinary tract infection. Symptoms such as fever, weight loss, and night sweats also are not unusual.

A variety of renal syndromes have been reported in patients with immunosupression. These can be either acute or chronic kidney disease including electrolyte abnormalities. Renal impairment from opportunistic infections and drugs used in these patients has also been reported. A broad spectrum of renal diseases affecting glomerular, tubular and interstitial tissues had been documented in immunocompromised patients especially HIV infected patients. Most of the renal manifestations represent complications of concurrent infections in a severely immunocompromised host, or side effects of the plethora of treatments required to manage these patients. The renal related presentations except for hypertension and oedema are consistent with clinical presentations in renal disease in immunocompetent patients, however severity varies with the degree of immunosuppression. Hypertension and oedema were reported as not common in immunocompromised patients. The renal disease in these patients deteriorates faster without intervention thus the need for early diagnosis and prompt intervention.

#### **9. Conclusion**

Immunocompromised patients are predisposed to a variety of clinical syndromes. The manifestations depend on the cause of immunosuppression, the degree of immunosupression, the endemic infections, the system or organ with predominant injury, and other associated diseases like Malignancies and infiltrative diseases. It is noteworthy that these patients may have atypical presentations. Thus there is need for surveillance and high index of suspicion of injuries/diseases in these patients to ensure early diagnosis and intervention.

#### **Author details**

188 Immunodeficiency

Urogenital tract infection has a different clinicoradiological presentation in immunocompromised patients, with predominance of systemic symptoms, multiple parenchymatous renal foci, and lower frequency of lesions of the collecting system. In the context of immunosuppression, Urogenital tract infection behaves as a severe bacterial

Many patients are asymptomatic. Symptoms that may occur include dysuria, urinary frequency and incontinence, flank pain, and fever. Confusion and delirium are often attributed to UTI, although without high fever or sepsis. Uncomplicated UTI is unlikely to cause serious central nervous dysfunction. The clinical signs and follow up of these infections were straight forward in half of the cases. However, in some patients, the infection is fulminant with progression to an abscess despite the use of antibiotics or is

Mycobacterial agents causing UTIs are less frequent in immunocompetent individuals; they are more common and severe in immunocompromised individuals. Extra pulmonary tuberculosis (EPTB) represents a progressively greater proportion of new cases and the genitourinary tract is the most common site of EPTB. The most common causative organism of kidney and urinary tract tuberculosis is the Mycobacterium tuberculosis, and occasionally Mycobacterium bovis can also be responsible. Mycobacterium tuberculosis (MTB) has an important impact on kidney transplant recipients, particularly during the first year after surgery. Tuberculosis of the urinary tract is easily overlooked. Symptoms that sometimes occur include back, flank and suprapubic pain, hematuria, frequency, and nocturia. These might also suggest conventional bacterial urinary tract infection. Symptoms such as fever,

A variety of renal syndromes have been reported in patients with immunosupression. These can be either acute or chronic kidney disease including electrolyte abnormalities. Renal impairment from opportunistic infections and drugs used in these patients has also been reported. A broad spectrum of renal diseases affecting glomerular, tubular and interstitial tissues had been documented in immunocompromised patients especially HIV infected patients. Most of the renal manifestations represent complications of concurrent infections in a severely immunocompromised host, or side effects of the plethora of treatments required to manage these patients. The renal related presentations except for hypertension and oedema are consistent with clinical presentations in renal disease in immunocompetent patients, however severity varies with the degree of immunosuppression. Hypertension and oedema were reported as not common in immunocompromised patients. The renal disease in these patients deteriorates faster without intervention thus the need for early diagnosis

Immunocompromised patients are predisposed to a variety of clinical syndromes. The manifestations depend on the cause of immunosuppression, the degree of

infection, with bacteremia and visceral metastatic foci.

unusual because of the pathogens isolated.

weight loss, and night sweats also are not unusual.

and prompt intervention.

**9. Conclusion** 

Umezurike Hughes Okafor *Enugu State University Teaching Hospital, Parklane, Enugu, Nigeria* 

#### **10. References**


Ping Zhang, Gregory J. Bagby, Kyle I. Happel, Caroline E. Raasch, Steve Nelson. Alcohol Abuse, Immunosuppression, and Pulmonary Infection Current Drug Abuse Reviews, 2008, Vol. 1, No. 1

**Chapter 8** 

© 2012 Sorensen et al., licensee InTech. This is an open access chapter distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

© 2012 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

**Selective Antibody Deficiency** 

**with Normal Immunoglobulins** 

Ricardo U. Sorensen, Tammy Harvey and Lily E. Leiva

unresponsiveness to polysaccharide antigens is not unusual. [21, 25]

Specific antibody deficiency (SAD) is a common antibody immunodeficiency defined as a poor antibody response to unconjugated pneumococcal polysccharides present in the 23 valent pneumococcal vaccine (PPV23). Clinical manifestations of specific antibody deficiency include recurrent sinopulmonary infections, such as sinusitis, otitis media, bronchitis, and pneumonia. All immunoglobulin concentrations, including IgG subclasses, are normal, and antibody response to protein antigens (eg, tetanus toxoid, diphtheria toxoid) and the conjugate H influenzae b vaccine are also normal in most patients. [11, 44, 56] In some patients with SAD, the response to the pneumococcal conjugate vaccines (PCV7,

SAD was first reported in a small group of patients in the early 1980s. [6, 46] The widespread use of pneumococcal immunization to assess antibody responses has revealed that specific

The vast majority of SAD patients have a deficiency of specific antibodies to polysaccharides but normal antibodies to protein antigens, resembling the developing immunologic status of human newborns and infants. Infants readily produce antibodies against vaccine proteins but fail to respond to most vaccine polysaccharides until approximately two years of age. In some patient with early onset SAD, this condition may represent a delayed maturation of

SAD is also found in association with many primary and secondary immunodeficiencies. An association of SAD with IgG subclass deficiencies, particulary IgG2 deficiencies, has been described. [9] IgG2 subclass deficient patients have antibody responses to a restricted number of polysaccharides in the PPV vaccine. Frequently, these patients also have poor immunological memory, with IgG antibody titers decreasing to pre-immunization levels

and reproduction in any medium, provided the original work is properly cited.

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/51709

PCV10, and PCV13) is also normal.

the immune response to polysaccharides.

within 6 to 12 months. [51]

**1. Introduction** 


#### **Chapter 8**
