PubMed Health⌕ Search

Biomedical subjects

Y Cesur

Publications and source records attributed to Y Cesur.

15 recordsLinked to original sources

A case of Allgrove (Triple A) syndrome associated with renal ectopia.

Allgrove syndrome (triple-A syndrome) is an autosomal recessive disorder characterized by adrenocorticotropin hormone-resistant adrenal insufficiency, achalasia and alacrima. Aside from the classic features of the syndrome, several abnormalities including mainly neurological abnormalities have been reported in the syndrome. Herein, we presented a case of Allgrove syndrome associated with left renal ectopla. To the best of our knowledge renal abnormality in Allgrove syndrome has not been reported in the literature until now. We think that ectopic kidney diagnosed in our patient is coincidental because the incidence of renal ectopia is high, approximately 1 in 900 in population.

Adrenal Insufficiency↗

A case of Melkersson-Rosenthal syndrome associated with Ehlers-Danlos syndrome.

Melkersson-Rosenthal syndrome (MRS) is characterized by the triad of recurrent facial palsy, lingua plicata, and facial edema. Herein, we report a case of MRS associated with Ehlers-Danlos syndrome due to rare presentation. To the best of our knowledge only one case of MRS associated with Ehlers-Danlos syndrome has been reported in the literature until now.

Child↗

Immunoglobulin subgroups in children with febrile seizures.

BACKGROUND: The aim of the present study was to determine whether or not there was a role for immunoglobulin (Ig) or IgG subgroups in the pathogenesis of febrile seizures (FS). METHODS: Serum levels of IgA, total IgG, IgM, IgE, IgGI, IgG2, IgG3 and IgG4 were measured in 34 children with FS and in 37 healthy children used as a control group. Both patients and controls were divided into two groups according to age (group I, 6-24 months; group II, 25-72 months). RESULTS: Compared with controls, mean IgG4 levels in patients were found to be decreased in both groups I and II (group I: 95 +/- 14 vs 57 +/- 5, respectively, P = 0.01; group II: 178.5 +/- 38.5 vs 65.1 +/- 24.5, respectively, P < 0.01), while mean IgG2 levels were found to be decreased only in group II patients (170 +/- 16 vs 103 +/- 22; P < 0.05). CONCLUSIONS: The results of the present study suggest that Ig subclass deficiencies may be responsible for the infections connected with FS or that they may be related to the pathogenesis of FS in some children.

Child↗

Assessment of bone ages: is the Greulich-Pyle method sufficient for Turkish boys?

BACKGROUND: The Greulich-Pyle (GP) Atlas of skeletal maturation has been prepared in white children who born between 1917 and 1942 in the USA, and is frequently used for assessment of skeletal maturity. In this study, we investigated whether or not the GP method is sufficient for Turkish children for the determination of the skeletal age. METHODS: Plain radiographies of left hands and wrists of 225 healthy boys between 7 and 17 years of age were taken. Pubic hair (PH) stages of boys were determined by using the Tanner criteria. Mean chronological ages and mean skeletal ages according to GP Atlas were compared for each age groups and each PH stage. RESULTS: Mean skeletal ages were delayed 0.61, 0.72, 0.54, 0.39, 0.25, 0.39, and 0.32 years than the mean chronological ages in the 7-13 years age groups, respectively, and advanced 0.13, 0.01, 0.89, and 0.52 years in the 14-17 years age groups. In PH stages 1, 2, and 3, mean skeletal ages were delayed 0.67, 0.51 and 0.40 years than the mean chronological ages, respectively. In PH stages 4 and 5, mean skeletal ages were advanced 0.66 and 0.76 years than mean chronological ages. CONCLUSION: The results suggest that Turkish boys may have a different tempo of skeletal maturation during pubertal development from that of American children which GP standards were derived. Therefore, GP Atlas is not completely applicable to Turkish boys but can be used with some modification.

Adolescent↗

A case of infantile cortical hyperostosis.

Infantile cortical hyperosteosis (ICH) is usually a self-limited disease of infancy with bony changes, soft tissue swelling, fever, irritability, decreased appetite, and decreased movement of the affected bones. Its description in isolated patients or in multiple members of families suggests the existence of two different forms, namely a sporadic form and a familial form with incomplete penetrance. In this article, we report a 2.5-month-old girl with ICH of sporadic form, due to unusual presentation.

Child, Preschool↗

Bilateral galactocele in a male infant: a rare cause of gynecomastia in childhood.

A galactocele is a rare benign breast lesion usually occurring in females during or following lactation. These lesions are a rare cause of breast enlargement in infants and children. In this article we present a 10 month-old boy who was admitted with a two-month history of bilateral progressive breast enlargement, and diagnosed as having galactocele. Our purpose was to emphasize the importance of galactocele as a benign condition in the differential diagnosis of gynecomastia in childhood.

Breast Diseases↗

Cutaneous anthrax in eastern Turkey.

Anthrax, caused by the spore-forming bacterium Bacillus anthracis, is rarely seen in industrial nations but is common in developing countries. Cutaneous anthrax (CA), the most common form of the disease, accounts for 95% of cases and usually develops on exposed sites. This study reviews the clinical and laboratory findings of 21 patients diagnosed with CA during 2 separate epidemics in the Van region of Turkey. All patients had a history of direct contact with infected cattle. The patients, aged 1.5 to 64 years, included 13 females and 8 males. Of the patients, 9 were 15 years or younger. Skin lesions were localized on the hands and fingers in 15 patients, on the face in 3 patients, on the face and finger in 1 patient, on the chest and finger in 1 patient, and on the eyelid in 1 patient. Gram-positive bacillus were noted on Gram stains of material obtained from skin lesions in 2 patients. All but one patient was successfully treated with penicillin; the unresponsive patient was treated with cefuroxime and required plastic reconstructive surgery because of a skin defect on the eyelid.

Adolescent↗

An analysis of seven infants with Brachmann-de Lange syndrome, of whom two identical twin sisters.

An analysis of seven infants with Brachmann-de Lange syndrome, of whom two identical twin sisters: Brachmann-de Lange syndrome (BDLS) is characterized by typical facial features, intrauterine growth retardation, short stature, microbrachycephaly, hirsutism and limb anomalies. Here, we reviewed the findings of seven infants with BDLS, of whom two were identical twin sisters of normal parents. All of the infants' parents were normal, and no consanguinity between the parents was noted although the ratio of consanguineous marriages is very high (21.1%) in Turkey. It is well known that most cases of BDLS are sporadic, some cases of this disorder are inherited in an autosomal dominant trait. Our findings suggested that familial cases of BDLS were infrequent, and vast majority of cases appeared to be sporadic and the occurrence of the syndrome in the identical twin sisters of normal parents was also thought a heterogeneity in this condition, overlapping with other conditions and syndromes as mentioned by Fryns et al.

De Lange Syndrome↗

Serum immunoglobulins and immunoglobulin G subclasses with recurrent wheezing.

In this study serum immunoglobulins (Ig) and IgG subclasses were measured in 42 patients (ranging 9 month-6 year) with recurrent wheezing and in 37 healthy children determined the relationship between serum Igs and recurrent wheezing. Patients were divided into two groups according to the age [9 month-2 year (n: 15), and 2-6 year (n: 27)]. In the patients placed in 9-24 month age group, serum IgG4 level was found to be lower than controls (p < 0.05). But there was not a significant difference in mean serum concentrations of total IgG, IgA, IgM, IgE, IgG1, IgG2 and IgG3 subclasses between the groups (P > 0.05). In the 25 month-6 year age group the mean IgE level was increased compared to the control while IgG3 and IgG4 levels were decreased (p < 0.05). On the other hand, in the 9-24 month age group there was no significant difference between the patients and controls for IgG subclasses deficiency (P > 0.05). However, significant difference in IgG subclasses deficiency was present between the patients and controls in the 25 month-6 year group (P < 0.001). In conclusion, our findings suggest that wheezing in childhood may be associated with low IgG3 and/or IgG4, and in older children high IgE level may be a part of pathogenetic mechanism in patients with recurrent wheezing.

Child↗

Urinary tract infection and antibiotic susceptibility in malnourished children.

In this study, 31 (30%) cases of urinary tract infection (UTI) of 103 patients with malnutrition, who were admitted to our hospital, were investigated prospectively. Our purpose was to determine the frequency of UTI, species of bacteria caused to infection and their antibiotic susceptibility in infants with malnutrition. The mean age of the patients with UTI was 11.5+/-7.6 months (ranging 50 days-30 months). The main symptoms were fever, vomiting, diarrhea, cough, and seizures. The mean body weight was 5.8+/-1.9 kg (2-10 kg), and height was 67.5+/-7.8 cm (53-85 cm). Seven of them had mild, 11 had moderate, and 13 had severe malnutrition. The most common isolated microorganism from urine cultures was Escherichia coli (54.8%). Most strains of Escherichia coli were resistant to co-trimoxazole (82.3%), ceftriaxone (17.6%), cefotaxime (17.6%), and ciprofloxacine (17.6%), but none of them were resistant to gentamicin. In conclusion, we would like to emphasize that UTI predominantly by gram negative microorganisms are frequent in the infants with malnutrition, and these microorganisms are mostly resistant to co-trimoxazole which is used commonly in practical medicine and prophylaxis.

Child, Preschool↗

Triple A syndrome mimicking cystic fibrosis.

We report a 2-8/12 year-old male who presented with symptoms resembling cystic fibrosis (failure to thrive, developmental delay and recurrent diarrhea) and had elevated sweat chloride concentration. Mucosal hyperpigmentation led to the diagnosis of adrenal insufficiency which was ultimately shown to be a component of triple A syndrome (achalasia, alacrima, adrenal insufficiency). Elevated sweat chloride concentration normalized after initiation of adrenal replacement therapy. We suggest that non-CF conditions causing elevated sweat chloride concentration should be considered in patients with atypical findings or who do not have objective evidence of pulmonary or exocrine pancreatic disease.

Adrenal Glands↗