Unusual Clinical Presentation of Hodgkin Lymphoma in a Child: Both Spinal Cord Compression and Hydronephrosis.

BACKGROUND: Hodgkin lymphoma (HL) is predominantly a nodal disease with extranodal presentation being uncommon. Presentation with neurological symptoms is not uncommon in adult patients with HL. Subdiaphragmatic involvements are less common especially in childhood. In the literature, there has been no case which presented with both spinal cord compression and bilateral hydronephrosis in pediatric patients with HL. OBSERVATION: We report a 9-year-old boy diagnosed with HL who presented with bilateral hydronephrosis and epidural involvement. CONCLUSION: Differential diagnosis of abdominal mass in patients presenting with spinal cord compression and/or hydronephrosis should include HL. Retrograde J ureteral stenting is the treatment of choice for malignant ureteral obstruction.

Efficacy of anakinra in a patient with systemic amyloidosis presenting as amyloidoma.

Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. We report a case of a soft tissue amyloidoma in the abdomen of a 16-year-old girl who was diagnosed as having systemic amyloidosis. A girl aged 16 years was referred to our hospital with a pre-diagnosis of a retroperitoneal mass documented with abdominal ultrasonography and tomography. A laboratory examination revealed nephrotic syndrome. She underwent surgery for a complete resection of the lesion. A histopathologic examination with Congo red and crystal violet dyes verified the diagnosis of amyloidoma. An immunohistochemical study for amyloid A protein was positive. A renal biopsy was also compatible with AA amyloidosis. A detailed search for the etiology of systemic amyloidosis revealed heterozygous mutation in the Mediterranean fever gene. Treatment with colchicine and anakinra were started with the diagnosis of familial Mediterranean fever because the other causes of secondary amyloidosis were ruled out. After 3 months of anakinra treatment, the laboratory findings returned to normal and excessive proteinuria disappeared. In countries where FMF and other autoinflammatory diseases are prevelant, systemic amyloidosis should be kept in mind in the differential diagnosis of children who present with nephrotic syndrome and abdominal mass. Taking previously reported cases and our case together, it appears that anti-interleukin-1 treatment represents a promising new approach in a subset of patients with systemic amyloidosis secondary to autoinflammatory diseases.

A newborn with antenatal testis tortion.

Testis tortion in the newborn (especially antenatal testis tortion) is observed very rarely and constitutes 10-12% of childhood testis tortions. In testis tortion, firm and painless testicular tissue is palpated on physical examination. Doppler ultrasonography is a sensitive method in the diagnosis. In cases of neonatal testis tortion, the testis can be saved with appropriate surgical exploration in only 0-5% of the cases. Here, a newborn with antenatal testis tortion who underwent orchiectomy in the first day of life was presented.

Traumatic lumbar artery pseudoaneurysm: the role of CT angiography in diagnosis and treatment.

Pseudoaneurysm of lumbar artery is a rare and late complication of penetrating trauma. We report the Doppler ultrasonography and multidetector computed tomography angiography findings and endovascular embolization treatment of a pseudoaneurysm of lumbar artery in a 14-year-old girl following a gunshot wound.

The right-sided aortic arch in children with esophageal atresia and tracheo-esophageal fistula: a repair through the right thoracotomy.

PURPOSE: The management of the esophageal atresia and tracheo-esophageal fistula (EA/TEF) with right-sided aortic arch (RAA) is controversial. The preoperative diagnostic techniques may fail to show RAA associated with EA/TEF. Surgeon may need to make a decision to change the side of thoracotomy. The aim of the current study was to evaluate the possibility of preoperative diagnosis of RAA and the primary anastomosis through right chest. METHODS: A retrospective review was performed in EA/TEF patients between February 2001 and 2008. A total of 79 patients (35 female, 44 male) with EA/TEF were reviewed. Eleven (13%) patients (5 female, 6 male) had an RAA. Echocardiography was performed in 10 of 11 patients with RAA. The chest was accessed through the right side in all patients. RESULTS: The incidence of RAA was found to be higher in our study than previous studies (13%). Right thoracotomy was performed successfully in all patients. Three patients died due to multiple congenital anomalies and 1 patient due to bleeding postoperatively. Five of 10 had normal echocardiography findings. Only one patient with RAA has been successfully diagnosed by preoperative echocardiographic examination. Seven patients had no complication after operation. Their follow-up was uneventful. CONCLUSION: Preoperative recognition of RAA with echocardiography is unlikely in patients with EA/TEF but the presence of RAA does not decrease the success rate of EA/TEF repair through the right thoracotomy.

Establishment of interdisciplinary child protection teams in Turkey 2002-2006: identifying the strongest link can make a difference!

OBJECTIVES: The University of Iowa Child Protection Program collaborated with Turkish professionals to develop a training program on child abuse and neglect during 2002-2006 with the goals of increasing professional awareness and number of multidisciplinary teams (MDT), regional collaborations, and assessed cases. This paper summarizes the 5-year outcome. METHODS: A team of instructors evaluated needs and held training activities in Turkey annually, and provided consultation when needed. Descriptive analysis was done via Excel and SPSS software. RESULTS: Eighteen training activities were held with 3,570 attendees. Over the study period, the number of MDTs increased from 4 to 14. The MDTs got involved in organizing training activities in their institutions and communities. The number of medical curriculum lectures taught by MDTs to medical students/residents, conferences organized by the MDTs, and lectures to non-medical professional audiences increased significantly (R(2)=91.4%, 83.8%, and 69.2%, respectively). The number of abuse cases assessed by the MDTs increased by five times compared to pre-training period. CONCLUSIONS: A culturally competent training program had a positive impact on professional attitudes and behaviors toward recognition and management of child abuse and neglect in Turkey. The need to partner with policy makers to revise current law in favor of a greater human services orientation became clear. PRACTICE IMPLICATIONS: Pioneers in developing countries may benefit from collaborating with culturally competent instructors from countries with more developed child protection systems to develop training programs so that professional development can improve recognition and management of child abuse and neglect.

Coin ingestion in children: which size is more risky?

AIM: Because of economic inflation, different-sized coins are in circulation in our country. The coin ingestion and retention in the esophagus are common problems in childhood. We evaluated the patients with coins retained in the esophagus and the impact of the size of the coins on lodgment. PATIENTS AND METHODS: Sixty-two children with a history of coin ingestion and a chest X-ray with retained coin in the esophagus were evaluated. Patients' age, sex, type of the ingested coin, and localization of coin were recorded. The size of all coins was measured. All coins were removed either directly with a Magill forceps or with the aid of an esophagoscope from the esophagus under general anaesthesia. RESULTS: There were 27 male and 35 female patients with coin lodgment (median age, 4 years; range, 1-13). Forty-five patients (73%) ingested a coin with a diameter between 23.45 and 26.00 mm. In the remaining 17 patients (27%), the coins had a diameter between 17.00 and 20.90 mm or between 26.85 and 28.00 mm. Fifty coins were at the upper esophagus, eight coins were in the middle esophagus, and 4 patients had a coin in the distal esophagus. There was a positive correlation between the diameter of coin and age of the patient (r = 0.415 and P < 0.001). CONCLUSIONS: Coin ingestion is rather common among childhood and its treatment may require an endoscopic approach. Most retained coins had a diameter between 23.45 and 26.00 mm. We think we could redesign our coins so that they would either be too big to ingest or so small they would always pass spontaneously.

Biochemical and histopathologic effects of omeprazole and vitamin E in rats with corrosive esophageal burns.

The aim of the study reported here was to evaluate the biochemical and histopathologic effects of omeprazole and vitamin E in rats with corrosive esophageal burns. A total of 144 Wistar Albino rats were divided into 12 experimental groups (12 rats per group) and used in an animal study. Group I rats were given a laparotomy and received no treatment (control group), while groups II, III and IV received a laparotomy and were treated with omeprazole, vitamin E or omeprazole/vitamin E, respectively. Groups V-XII rats received a laparotomy and were given a caustic acid burn through acid instillation (1 ml caustic 10% sulphuric acid; groups V-VIII) or alkali instillation (corrosive 10% sodium hydroxide solution; groups IX-XII) into the isolated esophageal segment via a 22-Fr cannula for 2 min. Each group of rats subjected to caustic burn received either no treatment (groups V and IX) or were treated with omeprazole, vitamin E or omeprazole/vitamin E, respectively (remaining six groups). Omeprazole (20 mg/kg) or vitamin E (10 mg/kg) was administered to the rats intraperitoneally or intramuscularly, respectively. Seventy-two rats (50% of each group, n = 6) were killed immediately after the experimental treatment (acute phase). The remaining rats were kept under standard conditions for 21 days (late phase) before being killed. The distal esophageal segments were harvested from all animal and used in histopathologic and biochemical analyses. Compared to the controls (no caustic burn), rats receiving only the acid or alkali installation (and no subsequent treatment) had the highest mean malondialdehyde (16.9 and 15.8 micromol MDA/g protein, respectively) and hydroxyproline (5.9 and 5.7; mg HP/g wet tissue) levels of all groups. In comparison, rats treated with acid + omeprazole and/or vitamin E had relatively lower MDA (12.9 and 11.6 micromol/g protein, respectively) and HP levels (4.3 and 4.08 mg/g wet tissue, respectively). Similarly, rats treated with alkali + omeprazole and/or vitamin E had low levels of MDA (13.9 and 11.7 micromol/g protein, respectively) and HP (4.3 and 4.4 mg/g wet tissue, respectively). The glutathione (GSH) levels of acid-only- or alkali-only-treated rats were lower than those found in omeprazole- and/or vitamin E-treated rats. Based on these results, we conclude that vitamin E and omeprazole affect the biochemical and histopathologic parameters in rats receiving corrosive esophageal burn from acid and alkali. The effect of both substances was slightly greater in the acid-treated groups.

Balloon-assisted single-port thoracoscopic debridement in children with thoracic empyema.

PURPOSE: In this study, we evaluated the results of a balloon-aided single-port thoracoscopic debridement of late-stage thoracic empyema in children. PATIENTS AND METHODS: We retrospectively reviewed age, gender, duration of prehospital illness, physical findings, surgical interventions, and the morbidity in 12 children with late-stage parapneumonic empyema. The diagnosis of pleural effusion was confirmed by a thoracocentesis before thoracoscopy. A balloon connected to a 12 F feeding tube was inserted into the thoracic cavity and inflated with air before the enterance of the thoracoscope. By this maneuver, a cavity was formed just under the enterance point. Thereafter, a routine debridement and chest irrigation was performed by thoracoscopy. Only one port was inserted in all but 1 patient, and the telescope was used as a dissecting tool. A thorax tube was inserted through the port site at the end of the procedure and left for the drainage. RESULTS: The main symptoms of the patients were dyspnea, cough, and fever. The empyema was located on the right hemithorax in 5 patients and on the left side in 7 patients. A second port was necessary to enhance the dissection in 1 case. The chest tube was removed within 3-30 days (median, 11 days) after the surgical approach. No complication directly related to the procedure was seen. The only problems postoperatively were a self-limited and spontaneously resolved bronchopleural fistula in 4 patients, and we had to perform an additional thoracoscopy to resolve the remaining intrapleural adhesions in 1 child. CONCLUSIONS: Thoracoscopic debridement in patients with late-stage thoracic empyema may be very beneficial, and this treatment method may provide any further thoracotomy. A balloon inflated in the thoracic cavity may achieve a wider field of vision for thorascopic surgery, and single-port thoracoscopy is sufficient and safe for the dissection.

Is there a hidden mortality after one-stage transanal endorectal pull-through for patients with Hirschsprung's disease?

One-stage transanal pull-through (TAP) has become a standard definitive procedure for the treatment of Hirschsprung's disease (HD). Short-term results of this operation seem to be excellent, but long-term outcome is still obscure. We evaluated the morbidity and mortality of our patients with one-stage TAP, and we reviewed the literature. We performed a TAP without a colostomy in 21 patients with HD. The primary outcome measures are age, sex, complications during surgery, enterocolitis (EC) attacks after surgery, postoperative stooling problems and mortality. All patients were called over telephone, and their clinical and functional outcomes were obtained. Case series of TAP in the literature were also reviewed in terms of postoperative problems. Twenty-one patients with full thickness rectal biopsy-proven HD underwent one-stage TAP. Average follow-up was 28 months. One early postoperative EC and three more late attacks of EC were observed. All survived patients had normal bowel habits. Three patients had perianal excoriations, three patients soiling, seven cases required anal dilatations and four patients experienced a diarrhea after surgery. We have been informed that four patients died after discharge from hospital. Two of them were a sudden death (one patient had metabolic problems, the other might have had an EC attack). The cause of death of one patient with an associated Down syndrome was a severe pneumonia, and one other case died of a septic shock of unknown etiology. None of these patients had a diarrhea or abdominal distention, which could have been an evidence of an EC attack prior to their deaths. We observed similar fatal cases, when reviewed the published series in the literature. There might be a hidden mortality within the long-term period after TAP for HD. Therefore, we recommend a close follow-up for all patients with any associated health problem and those from low socioeconomic regions after one-stage pull-through.

Epidermal growth factor and bombesin act synergistically to support intestinal adaptation in rats with massive small bowel resection.

Intestinal adaptation is the most important event in short bowel syndrome following a massive small bowel resection. Effects of various growth factors and their synergism have been well documented in intestinal adaptation. This study aimed to compare the effect of two different trophic agents, epidermal growth factor (EGF) and bombesin (BBS), on intestinal adaptation following massive intestinal resection. Sprague-Dawley male rats were assigned to one of four groups after a 75% small bowel resection. Either EGF (90 microg/kg), BBS (10 microg/kg), EGF+BBS, or bovine serum albumin (BSA) were injected subcutaneously three times a day. The animals were killed 10 days after the operation. Weight loss and morphologic parameters such as mucosal thickness, villus height, crypt depth, villus-to-crypt ratio, and muscularis propria height were measured. In the EGF+BBS group, mucosal thickness was found to be significantly increased compared with the other study groups (p<0.05). Similarly, villus height was significantly increased only in the EGF+BBS group (p<0.05). In the BBS group, both villus height and mucosal thickness showed a slight increase, but the values were not statistically significant compared with the vehicle-treated group. There were no significant differences in any of the remaining parameters between the groups. The results of this study indicate that the gut hormones EGF and BBS act synergistically in facilitating the adaptive response of the remnant ileum to massive intestinal resection.