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Biomedical subjects

A Hiçsönmez

Publications and source records attributed to A Hiçsönmez.

At least 19 recordsLinked to original sources

A very unusual presentation of Niemann-Pick disease type B in an infant: similar findings to congenital lobar emphysema.

The main features of Niemann-Pick disease type B (NPD-B) are enlargement of the liver and spleen, and mild pulmonary involvement. Recurrent respiratory tract infection and progressive decline in pulmonary function are major contributors to morbidity and mortality in this patient group. Massive pulmonary involvement in early life is extremely rare. The most common finding on chest X-rays of NPD-B patients is reticular or nodular infiltration of the lungs. This article describes a very rare presentation of NPD-B in an infant who had suffered recurrent respiratory tract infections. Massive emphysema and marked infiltrative parenchymal changes (infiltration of the parenchyma) were initially attributed to congenital lobar emphysema and its compressive effects. However, NPD was suspected when a lung biopsy showed foamy cells and sea-blue histiocytes were detected in a bone marrow biopsy. The definitive diagnosis was established with an enzyme study for sphingomyelinase.

Diagnosis, Differential↗

Three-dimensional conformal breast irradiation in the prone position.

The prone position can be used for the planning of adjuvant radiotherapy after conservative breast surgery in order to deliver less irradiation to lung and cardiac tissue. In the present study, we compared the results of three-dimensional conformal radiotherapy planning for five patients irradiated in the supine and prone position. Tumor stage was T1N0M0 in four patients and T1N1M0 in one. All patients had been previously submitted to conservative breast surgery. Breast size was large in three patients and moderate in the other two. Irradiation in the prone position was performed using an immobilization foam pad with a hole cut into it to accommodate the breast so that it would hang down away from the chest wall. Dose-volume histograms showed that mean irradiation doses reaching the ipsilateral lung were 8.3+/-3.6 Gy with the patient in the supine position and 1.4+/-1.0 Gy with the patient in the prone position (P = 0.043). The values for the contralateral lung were 1.3+/-0.7 and 0.3+/-0.1 Gy (P = 0.043) and the values for cardiac tissue were 4.6+/-1.6 and 3.0+/-1.7 Gy (P = 0.079), respectively. Thus, the dose-volume histograms demonstrated that lung tissue irradiation was significantly lower with the patient in the prone position than in the supine position. Large-breasted women appeared to benefit most from irradiation in the prone position. Prone position breast irradiation appears to be a simple and effective alternative to the conventional supine position for patients with large breasts, since they are subjected to lower pulmonary doses which may cause less pulmonary side effects in the future.

Breast Neoplasms↗

A comparative histopathologic and immunohistopathologic evaluation of cremaster muscles from boys with various inguinoscrotal pathologies.

AIM: It has been hypothesized that the cremaster muscle (CM) might play a part in the pathogenesis of various inguinoscrotal pathologies such as undescended testis and hernia. A prospective study was carried out to determine if CM of boys with hydrocele, inguinal hernia, and undescended testis reveal any histopathological and immunohistopathological alterations. METHODS: Samples of CM from 29 patients presenting with inguinal hernia (15), undescended testis (9), and hydrocele (5), and CM from autopsies of boys without inguinal pathology (2), and samples of internal oblique muscles from patients undergoing laparotomy (3) were obtained. The biopsies were frozen in isopentane-cooled liquid nitrogen, cut in 6 micron sections and stored at -80 degrees C until processed. Sections were stained by hematoxylin-eosin, modified Gomori-trichrome, PAS, Oil Red-O, NADH, SDH and COX. All specimens were evaluated for seven parameters including variation in fibre size, presence of central nucleus, endomysial fibrosis, inflammation, presence of basophilic fibres, increase in perimysial connective tissue, and variation in fibre size between fascicules. The specimens were also evaluated for beta-spectrin, laminin alpha-1 chain, laminin alpha-2 chain, 43 kd distroglycan, and fetal myosin by immunofluorescence. The presence of each parameter was compared, individually and in combination, according to the ages and underlying pathologies. RESULTS: None of the internal oblique muscles were positive for any of the seven parameters. Only one of the two CM obtained during autopsy revealed a slight variation in fibre size. However, fibre size variation and increase in perimysial connective tissue were found in all but one CM from a patient with hernia. The presence or absence of parameters did not differ according to age. Comparison of the groups with inguinal hernia and undescended testis for each of the individual parameters did not reveal significant differences. However, the presence of four or more parameters in each CM was more commonly encountered among patients with undescended testis compared to patients with hernia (p < 0.05). The CM of patients with hydrocele suggested similar findings to patients with inguinal hernia. All of the specimens, regardless of origin, revealed normal sarcolemmal and basal laminal stainings, and fetal myosin was expressed in only two specimens which were obtained from an internal oblique muscle and the CM of a patient with an inguinal hernia. CONCLUSION: The CM of patients with inguinal hernia, hydrocele, and undescended testis reveal histopathological alterations. Furthermore, CM from patients with undescended testis reveal more profound alterations. Whether these alterations reflect a primary muscle pathology or a defect in innervation or changes secondary to inguinoscrotal pathology requires further study.

Child↗

Surgical treatment of differentiated thyroid carcinoma in children.

INTRODUCTION: A retrospective clinical study was carried out to define the appropriate definitive operation for treating differentiated thyroid carcinoma (DTC) in children. METHODS: During a 24-year period between 1974 and 1997, 18 children younger than 16 years at presentation were histopathologically proven to have DTC in Hacettepe University Children's Hospital, Department of Pediatric Surgery. The clinical characteristics on admission, operative findings, procedures, operative complications, and follow-up results according to definitive operations were compared to find out the appropriate procedure. RESULTS: There were 15 female (83.3%) and 3 male (16.7%) patients yielding a female/male ratio of 5 and the average age being 11.6+/-2.1 years. The definitive operations were near total thyroidectomy, total thyroidectomy, near total thyroidectomy plus excision of the only palpable lymph nodes (EPLN), total thyroidectomy plus EPLN and total thyroidectomy plus modified radical neck dissection (MRND) in 3 (16.7%), 3 (16.7%), 1 (5.5%), 9 (50%), and 2 (11.1 %) patients respectively. Among the patients having undergone near total thyroidectomy, one had recurrences both in the thyroid bed and the cervical lymph nodes. In a patient undergoing almost total thyroidectomy and EPLN, recurrences involving thyroid bed, cervical lymph nodes and lungs were encountered. In 9 patients undergoing total thyroidectomy and EPLN 4 had cervical lymph node recurrences. Complications have included permanent hypoparathyroidism following total thyroidectomy and MRND. Additionally, operations for recurrences resulted in unilateral permanent recurrent laryngeal nerve palsies in two patients. CONCLUSIONS: Total thyroidectomy with evaluation of central compartment, entire jugular chain and region of lower spinal accessory nerve and removal of all identifiable lymph nodes seems to be the most appropriate surgical treatment for DTC encountered in children.

Adenocarcinoma, Follicular↗

Gastric outlet obstruction due to corrosive ingestion: incidence and outcome.

A retrospective clinical study was performed to determine the incidence, management, and outcome of gastric outlet obstruction (GOO) caused by caustic ingestion in children. Of 220 patients who sustained caustic substance ingestion and were treated at our unit between 1976 and 1996, 168 ingested alkaline substances; of these, 9 children (5.3%) developed GOO in addition to esophageal strictures. The remaining 52 patients ingested acid agents, and 2 of them (3.8%) presented with GOO without esophageal strictures. The overall incidence of corrosive GOO was 5% (n = 11). The mean age of the patients with GOO was 5.7 +/- 2.8 years (range 2-14) with a female:male ratio of 6:5. Sodium hydroxide (n = 6), potassium hydroxide (n = 3), and hydrochloric acid (n = 2) were the ingested caustic agents. The patients were subdivided into two groups according to serial endoscopic and radiologic findings: group I: moderate (dense superficial and spotty ulcerations with intact mucosa) mucosal injury with partial pyloric obstruction; and group II: severe (deep ulcerations, extreme hemorrhagic erosions, eschar formation with white plaques) mucosal injury with complete pyloric obstruction. Group I consisted of 5 patients who ingested alkali agents while group II included 6 who presented with ingestion of alkaline (n = 4) and acid (n = 2) agents. Surgical treatment included Billroth I (n = 6) operations performed in group II and Finney (n = 3) and Heineke-Mikulicz (n = 2) pyloroplasty procedures done in group I. All patients are alive without any complaints. Fiberoptic endoscopy should be the preferred method of evaluating a patient with ingestion of a corrosive agent. It determines the presence of injury and assesses the extent of damage, establishing the diagnosis and allowing therapy to be instituted immediately. Our experience revealed that substantial damage has occurred early after ingestion, and early surgical intervention has decreased the morbidity and mortality. The extent of the mucosal injury and status of the pylorus and antrum determined the type of surgical treatment. A Billroth I procedure recommended for severely injured mucosa with complete pyloric obstruction, and pyloroplasty for moderate mucosal injury associated with partially obstructed but still viable pylorus. In contrast to the current belief, alkali ingestion also has a high risk of corrosive gastric injury causing GOO, which should be considered during assessment of the injury. We emphasize that a detailed evaluation of radiologic and especially endoscopic findings is very important for determining the timing, necessity, and type of appropriate surgical treatment.

Alkalies↗

A previously unmentioned surgical observation in the treatment of intussusception.

Intussusception is one of the most common causes of bowel obstruction in infancy which may require surgical treatment. We have recently recognized a pathologically weakened linear area located on the wall of the reduced colon at operation. Thus, a retrospective study was conducted to evaluate the operative and pathological findings of the resected bowel segments for the treatment of intussusception. A pathologically weakened longitudinal linear area was encountered in five patients. This line was strikingly antimesenteric and under the taenia libera. The bowel wall was very thin and effaced on palpation along this whitish line and showed mucosal necrosis, disruption of the muscularis mucosa, and loss of some of the muscular tissue on microscopic examination. The line may result from compression of the inner layers of the bowel wall between the intussusceptum and the noncompliant taenia. Location on the antimesenteric border and under the taenia libera can be explained by local vascular compromise due to the distribution of the terminal arteries of the colon. Thus, the antimesenteric border as well as the mesenteric side should be checked carefully for a longitudinal weakened pressure line. The recognition of such a potentially dangerous weak line on the bowel wall indicates resection.

Child, Preschool↗

The preventive effect of heparin on stricture formation after caustic esophageal burns.

BACKGROUND/PURPOSE: Preventing thrombus formation after caustic esophageal ingestion has been proposed to have beneficial effects. Therefore, an experimental study was carried out to investigate the effects of heparin on the esophagus after caustic burns. METHODS: Caustic esophageal burns were produced in rats by irrigation with 50% NaOH as described by Liu. Rats were divided into four groups as follows: group A, animals with esophageal burns, received placebo and underwent autopsy 48 hours after caustic injury; group B, animals with esophageal burns, received subcutaneous heparin treatment and underwent autopsy 48 hours after caustic injury; group C, animals with esophageal burns, received placebo and underwent autopsy 28 days after caustic injury; group D, animals with esophageal burns, received subcutaneous heparin treatment for 7 days and underwent autopsy 28 days after caustic injury. Histopathologic evaluation was performed in all groups, and collagen content of esophageal sections was analyzed by determination of hydroxyproline levels. RESULTS: Submucosal vascular thrombosis was encountered in all group A animals but the submucosal venules and arterioles were patent in most of group B animals. Esophageal strictures did not develop in any of group D animals, although varying degree of esophageal stenoses were encountered in all animals of group C. The circumferences of the burned segment have been narrowed to 3+/-1 mm in group C rats. There was obvious collagen deposition in submucosa, and epithelial regeneration was not complete in group C rats. Submucosa and epithelial integrity seemed normal in group D animals. Hydroxyproline contents in group D were significantly lower compared with group C (P < .05). CONCLUSIONS: Heparin has ameliorating effects on stricture formation after caustic esophageal burn. Those effects may occur through possible anticoagulant, antithrombotic, and endothelial protective effects, and modifying effects of heparin on wound healing.

Animals↗

Biochemical predictors for differentiating intraperitoneal and extraperitoneal bladder perforation.

PURPOSE: A retrospective clinical study was performed to find out whether serum biochemistry alterations may serve to differentiate the traumatic bladder perforation to be either intraperitoneal or extraperitoneal. METHODS: Thirty-two children treated for traumatic bladder perforation between 1970 and 1997, inclusive, formed the study group. Patients were divided into two groups: intraperitoneal bladder perforation (IBP) and extraperitoneal bladder perforation (EBP) groups. The groups were compared with regard to age, sex, mechanism of injury, and hemodynamic parameters (blood pressure, hematocrit) at presentation (each group was further subdivided into two subgroups according to the duration between trauma and presentation as early (duration <24 hours) and late (duration >24 hours) presentation groups). The subgroups were compared with each other with respect to serum concentrations of urea, creatinine, sodium, potassium, and chloride. RESULTS: There were 21 boys and 11 girls with a mean age of 8.7+/-4.9 years. Mechanisms of injury were motor vehicle accidents (75%), falls (19%), and gunshot wounds (6%). There was no significant difference between the IBP and EBP groups with regard to age, sex, mechanism of injury and hemodynamic parameters at presentation. The biochemical parameters were found to be within normal range in the EBP group, whereas significantly higher levels of creatinine, potassium, and lower level of sodium were noted in IBP group regardless of presentation time. Significantly higher level of serum urea was recorded in a late presentation group of IBP patients. CONCLUSIONS: The authors emphasize that patients presenting with IBP are more likely to present with significantly higher levels of creatinine, potassium, and lower level of sodium compared with the patients with EBP regardless of presentation time. Increased level of serum urea concentration is a characteristic of IBP patients with late presentation. Biochemical alterations can be used to differentiate traumatic IBP and EBP in children with subtle physical examination and radiological findings.

Adolescent↗

Vaginal atresia and Bardet-Biedl syndrome association: a component or a distinct entity?

Bardet-Biedl syndrome is an autosomal recessive disorder. It is characterized by cardinal anomalies including retinal dystrophy, digital malformations, mental retardation, obesity, and hypogonadism. Recently, renal anomalies also are mentioned among the cardinal signs. Although association of genital anomalies among affected boys are well known, the association of vaginal atresia and other structural genital anomalies are not mentioned among the less-common manifestations of Bardet-Biedl syndrome in girls. Two girls with Bardet-Biedl syndrome presented with hematometrocolpos in the preadolescent period and vaginal atresia was diagnosed. After surgical treatment and extended hospitalization, uncontrolled sepsis resulted in progressive renal failure and death of both patients. Vaginal atresia is often delayed or missed in the early childhood period. In girls with Bardet-Biedl syndrome, vaginal atresia or other structural genital anomalies should be evaluated more systematically during the initial diagnosis of the syndrome. In infancy, the evaluation of a child with vaginal atresia also should include the differential diagnosis of Bardet-Biedl syndrome. Vaginal atresia may either form a component of the syndrome, or girls who present with vaginal atresia in addition to other components of Bardet-Biedl syndrome might form a distinct entity.

Abnormalities, Multiple↗

Abnormal prostatic utricle configuration in hypospadias and intersex patients.

A retrospective clinical trial was performed to evaluate the incidence, clinical characteristics, diagnosis and treatment methods of abnormal prostatic utricle configuration (APUC) in hypospadias and intersex patients. Forty-five hypospadias and 72 intersex patients in whom the configuration of prostatic utricle (PU) was evaluated by radiological and endoscopic investigations formed the study group out of 380 hypospadias and 160 intersex patients who were treated between 1975 and 1996. Enlargement of PU (EPU) was classified into four grades based on the radiological configuration. The term vagina masculina (VM) was used to define the presence of unregressed müllerian duct structures. The incidence of APUC (either enlarged PU: EPU or VM) was 13.3% (n = 6) and 16.6% (n = 12) in hypospadias and intersex patients, respectively. The incidence and grade of EPU increased significantly with the severity of hypospadias. Urinary infection, obstruction and enuresis were the most common symptoms in both groups. Medical treatment consisting of appropriate antibiotics associated with follow-up of the APUC by means of radiological and endoscopic investigations was carried out in 4 patients with Grade I EPU successfully. Surgical excision of the Grade II, III and/or VM through perineal (n = 5), transabdominal extravesical (n = 5) and perineal plus transabdominal extravesical (n = 3) approaches was done while one patient was lost for follow-up before treatment. We emphasize that surgical treatment of EPU and VM must aim at complete excision without damaging continence and sexual potency mechanisms. The selection of the surgical treatment method should be individualized according to the grade of APUC which must be defined by preoperative radiological and endoscopic investigations. The optimal surgical method is the one which allows the best visualization of the involved anatomy, facilitates accurate suture placement with complete removal of the EPU and VM and avoids inadvertent injury to the adjacent tissues. Guidance by urethral and utricular catheters is essential for safe and elegant dissection in all surgical methods.

Child↗

In-utero defecation: fact or fiction?

An experimental study was performed to investigate the gastrointestinal motility and in-utero defecation by radionuclide techniques. Forty-eight New-Zealand white rabbit fetuses at 25 days' gestation (fullterm, 31 to 32 days) were divided into two groups as A (n: 24) and B (n: 24). 0.1 ml of Technetium-99m (99mTC)-HIDA (a derivative of iminodiacetic acid) containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus, which had been exposed through the uterus. This procedure was used for all fetuses and additionally surgical closure of anus by a purse string suture was performed in Group B fetuses. After replacing the fetus and uterus into the abdomen, and beginning 1 hour after injection, a live fetus was killed each hour for 24 hours. Tissue samples from the reference organs (lung, heart, stomach, kidney, bladder), liver, meconium in the proximal, mid and distal bowel, and amniotic fluid were taken. The radioactivity of each sample was determined by a gamma counter and the percentage injected dose (uptake) per gram of tissue is calculated. 99mTc-HIDA was predominantly trapped by the liver via systemic circulation and excreted into the gastrointestinal tract in both groups through which it passed into the amniotic fluid only in Group A. The very low radioactivity levels detected in the amniotic fluid of Group B originated from urinary tract and indicated the in-vivo stability of 99mTc-HIDA, thus reliability of the experimental model. Intestinal transport of a radiopharmaceutical agent in both groups and demonstrated passage into the amniotic fluid only in Group A strongly suggest that fetal defecation is a physiologic event.

Amniotic Fluid↗

Ectopic gastric mucosa causing dysphagia due to strictures in a boy.

Ectopic gastric mucosa in the upper esophagus has been reported previously in neonatal autopsy series and encountered in adult esophagoscopies. Despite the usual asymptomatic course of the disease, symptomatic adults have been reported. However there is no report of a symptomatic child with ectopic gastric mucosa in the literature. A 12-year-old boy presenting with dysphagia due to strictures resulting from circular patches of ectopic gastric mucosa located in the mid esophagus is reported.

Choristoma↗

The use of eccentric circummeatal-based flap with combined limited urethral mobilization technique for distal hypospadias repair.

We report a suitable approach to the correction of subcoronal meatus for any variants and report our experience with this method. An eccentric circummeatal-based flap was used to treat 42 patients with hypospadias during a 4-year period. The position of the meatus was coronal in 18 patients (43 percent) and subcoronal and distal penile in 16 (38 percent) and 8 (19 percent) patients, respectively. In addition, 19 patients had marked ventriflexion. The mean length of the urethroplasty was 1.1 cm (ranging from 0.6 to 1.7 cm). In no instance was residual ventral curvature noted nor did the neourethra restrict the phallus during erection. The functional and cosmetic results were excellent, and in many cases the glans penis assumed a nearly normal shape and appearance. No patient required a second procedure to improve cosmesis. This is a convenient procedure for correction of distal hypospadias in which the urethral gap is no longer than 2 to 2.5 cm. The surgical repair does not depend on glanular or meatal variations or even if the patient had a previous operation. It is particularly suitable in cases that have marked fibrous chordee.

Adolescent↗

Systemic harmful effects of ingestion of household bleaches.

Household bleaches that contain sodium hypochlorite and sodium hydroxide are the common domestic agents used all around the world, which are frequently accidently ingested by children. Commercial household bleaches are known to have minor adverse effects when ingested, as being mucosal irritants, emetics and weak corrosives. However, our clinical observations of pneumonitis among household bleach ingested by patients without a history of aspiration foretell some possible systemic toxic effects of household bleaches. An experimental study on rats was designed to evaluate systemic effects of household bleaches that contain 4% sodium hypochlorite and less than 0.05% sodium hydroxide on lungs, livers, kidneys and intestines after 2, 4, 6, 12, 24 and 48 hours of administration via intragastric route. Prominent congestion and some interstitial mononuclear cellular infiltration were observed in the lungs, the livers and the kidneys of the rats after administration of household bleaches. Additionally, the lungs showed expansion of the alveolar spaces. While erosive changes were present in the stomachs, the intestines were normal. These histopathological changes were especially prominent at early periods of systemic administration. In the second part of the study, to assess whether these findings would hold for intravenous administration of household bleaches, another group of rats were given intravenous administration of household bleach and after 4 hours of intravenous administration of household bleach, the same histopathological changes above were observed in the lungs, kidneys and livers. The study indicates that household bleaches may have systemic effects prominent during early periods of ingestion, although the clinical importance remains to be clarified.

Animals↗

Combined use of steroid, antibiotics and early bougienage against stricture formation following caustic esophageal burns.

BACKGROUND: If an esophageal burn is diagnosed, the aim is to prevent stricture formation. The combined use of steroid, antibiotics and early bougienage (SAEB) is one of the currently used treatment protocols. The effect of SAEB treatment against stricture formation following caustic esophageal burn has been evaluated retrospectively. METHODS: Forty-nine children of 282 admitted with a history of caustic substance ingestion were found to have esophageal burns. Forty-nine children underwent treatment against stricture formation. SAEB was begun within 48 hours of ingestion. RESULTS: Eight children of 12 who ingested sodium hydroxide and five children of 20 who ingested acids developed strictures in spite of the therapy. Additionally two esophageal perforations were encountered in patients who ingested sodium hydroxide. CONCLUSIONS: This protocol has been found to carry a risk of perforation without preventing stricture formation after strong alkali ingestion. Therefore other treatment modalities for preventing strictures should be evaluated especially in children who have ingested products containing strong alkalis.

Anti-Bacterial Agents↗