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Tuesday, July 18, 2017

Case Study and Case Report 2017; 7(3): 65 - 75.

Salmanoglu E, Kurutas EB. Effects of 99mTc-MDP bone scintigraphy on
oxidative/nitrosative stress biomarkers in patients. Case Study Case Rep. 2017; 7(3): 65 - 75

ABSTRACT
We aimed to define the effects of Technetium-99m Methylendiphosphonate bone
scintigraphy on oxidative/nitrosative stress in patients after intravenous injection and
compared to healthy control group. Forty patients for bone scintigraphy and forty healthy
control group were included this study. The age range was similar in both groups (18-74
years). The blood samples were taken from patients 2 hour after intravenous injection of 20
mCi (740 MBq) Technetium-99m Methylendiphosphonate. The activities of catalase,
superoxide dismutase and malondialdeyhde levels were measured as oxidative stress
biomarkers and nitric oxide and nitrotyrosine levels were measured as nitrosative stress
biomarkers in patients and control group. There were no significant differences according to
both age and sex between two groups (p˃0.05). Activities of catalase and superoxide
enzymes and malondialdeyhde levels in patients were increased in patients compared to
controls (p<0.05). Also, nitric oxide and nitrotyrosine levels in patients were higher than
control group (p<0.05). Technetium-99m Methylendiphosphonate may cause both oxidative
and nitrosative stress in patients. Also antioxidant defense of the patients was higher
compared to healthy subjects possibly due to a compensatory response to ionising radiation in
the living cells and thereby protects the cells against oxidative/nitrosative damage.
Keywords: Bone scintigraphy, oxidative/nitrosative stress, catalase, superoxide dismutase,
malondialdehyde, nitric oxide, nitrotyrosine

Saturday, July 8, 2017

Case Study and Case Report 2017; 7(3): 53 - 64.

Mól N, Ptak K, Zasada M, Końska K, Pilch M, Knapp A, Kwinta P. Hunter Syndrome complicated by a communicating hydrocephalus and arachnoid cysts at a very young age. Case Study Case Rep. 2017; 7(3): 53 - 64.

ABSTRACT

Mucopolysaccharidosis type II (MPS II) is an X-linked recessive lysosomal storage disease caused by deficient activity of iduronate 2-sulfatase, which regulates  lysosomal catabolism of the glycosaminoglycans. As a consequence, undegraded dermatan and heparan sulfates accumulate in all the tissues and organs of the body, including the central nervous system, where they cause irreversible changes. Some of the neurological manifestations include behavioral problems, mental retardation or cognitive deterioration, ventriculomegaly, hydrocephalus, compressive myelopathy, seizures or hearing loss. This report describes an infant boy diagnosed with MPS II who presented with an acute communicating hydrocephalus and arachnoid cysts at 9 months of age. The patient was diagnosed with MPS II shortly after birth because of his positive family history. At that time he did not present any physical manifestation of the disease, all the imaging studies of the head, abdomen and cardiac function were normal. He was started on enzyme replacement therapy with Elaprase®, which he received every week at our Clinic. Such frequent visits allowed for an immediate diagnosis of sudden increase of the intracranial pressure and the timely surgical intervention. To the best of our knowledge, this is the first reported case of a patient with MPS II, who developed acute hydrocephalus requiring surgical implementation of a ventriculo-peritoneal shunt at such a young age.

Keywords: Hunter Syndrome, hydrocephalus, arachnoid cyst