A case of recessive dystrophic epidermolysis bullosa.
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Age Determination by Skeleton↗
Biomedical subjects
A Okten
Publications and source records attributed to A Okten.
Various clinical aspects of DIDMOAD (Wolfram) syndrome.
The association of juvenile diabetes mellitus (DM), diabetes insipidus (DI), optic atrophy (OA) and sensorineural deafness (D) is known as DIDMOAD or Wolfram syndrome. Aside from these four cardinal features, a wide variety of abnormalities of the nervous system, urinary tract and endocrine glands have been described in this syndrome. In this report, the clinical features of six patients with DIDMOAD syndrome are presented. All six patients had DM. Five of the six patients had DI, five OA and five displayed abnormal audiogram findings. In addition, two had goiter, two delayed puberty, one seizure and one mental retardation with depression attacks. Urinary tract dilatation was recorded in five patients. Four patients developed typical complications of DM. One of them had overt nephropathy and arthropathy despite the short duration of DM. In addition, this patient had diabetic retinopathy, which is considered to be rare in this syndrome.
Adolescent↗
The role of high-dose methylprednisolone therapy in paroxysmal nocturnal hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by intermittent hemolytic anemia. High-dose methylprednisolone (HDMP) was administered in two patients, eight- and 16-year-old females, with PNH. This drug produced a dramatic improvement in the hemoglobin level, leukocyte and platelet counts. No side effect was observed in either patient during the treatment period. The patients were followed up on an outpatient basis for six and 16 months. In conclusion, HDMP therapy for PNH appears to be more effective and safe than previously reported therapies.
Adolescent↗
The role of calcium channel entry blocker in experimental ischemia-reperfusion-induced intestinal injury.
The effect of verapamil on mesenteric ischemia reperfusion trauma was evaluated. Ischemia was performed with clamping a mesenterica superior for a period of 15 min. In 55 rats in histopathologically investigated group, the abdomen was opened 4 h after from ischemia and intestinal biopsies were performed. 55 rats were classified into 5 groups. In the first group 13 rats were chosen as control, in the second group 11 rats were chosen as SHAM control. Only midline incision was made for these animals without superior mesenteric artery clamping; in the third group (n = 10) intravenous verapamil (0.3 mg/kg) was given 15 min before ischemia; in the group 4 (n = 10) 10 min after ischemia; in the group 5 (n = 11) 10 min after reperfusion. There was statistically significant difference between group 2 and group 1 in injury severity (p < 0.05). Similarly, the injury severity of group 3 was statistically significant as compared to group 1 (p < 0.05). There was no significant change in severity of injury in group 4 and group 5 as compared to group 1 (p > 0.05). We concluded that verapamil was protective when administered during the ischemic period but did not exert this action if administered after established ischemia.
Animals↗
Asymmetric crying facies: an index of other malformations.
Congenital hypoplasia or absence of the depressor anguli oris muscle is a minor congenital anomaly causing asymmetrical crying facies (ACF). The interesting aspect of this abnormality lies in the frequently associated abnormalities. Cardiac, urogenital, musculoskeletal, respiratory and cervicofacial defects have been described in cases with ACF. Therefore it is suggested that ACF can be used as an index of other congenital malformations. We report three cases with ACF who had varied congenital anormalites.
Abnormalities, Multiple↗
Langerhans cell histiocytosis associated with recurrent pneumothorax: a case report.
Pulmonary involvement of Langerhans cell histiocytosis (LCH) is an uncommon but important cause of pulmonary fibrosis and honeycombing in young adults. Rarely, pulmonary LCH may be complicated by spontaneous pneumothorax. It may be isolated or associated with multiple organ involvement. We describe here a case of LCH with diabetes insipidus, skin lesions and pulmonary involvement in a 15-year-old boy. The case was complicated by four episodes of spontaneous pneumothorax with typical radiologic findings of pulmonary LCH. His presentation, radiologic findings, treatment and clinical course during three-years of follow-up are discussed.
Adolescent↗
Absorption of iron from grape-molasses and ferrous sulfate: a comparative study in normal subjects and subjects with iron deficiency anemia.
We compared the absorption of iron from grape molasses (GM) and ferrous sulfate (FS) using the post-absorptive serum iron increase method (non-radioactive). The study involved 56 subjects, aged 6-36 months. Group I consisted of 30 subjects with iron deficiency anemia (IDA) and group II, 26 non-anemic subjects. Each group was subdivided randomly into two equal subgroups to be given either GM or FS. The absorption of the iron from GM was monitored in 15 infants with IDA and in 13 non-anemic infants, and the absorption of iron from FS was tested in the rest of the subjects in each group. In those infants in each group given GM or FS, there was no significant difference in before-test values for serum iron (SI) and total iron binding capacity (TIBC) (p > 0.05). In the group with IDA, the mean after-test SI value in FS-given infants was higher and the mean TIBC value lower than those of GM-given infants (p < 0.05). However, in the non-anemic group, there was no significant difference in the mean after-test SI and TIBC values in either GM- or FS-given infants (p > 0.05). The mean increase of serum iron in GM-given infants with IDA was 27.0 +/- 13.4 micrograms/ dl and in FS-given infants, 60.6 +/- 17.0 micrograms/dl (p < 0.05). In the non-anemic group, the mean increase of serum iron of GM-given infants was 23.0 +/- 4.3 micrograms/dl, and that of FS-given infants, 23.8 +/- 10.0 micrograms/dl (p > 0.05). We determined that in non-anemic subjects, the absorption of iron from GM was comparable to that from FS. Furthermore, we believe that grape molasses is an effective iron source in preventing iron deficiency anemia in infancy.
Absorption↗