Two risk factors for hypozincemia in diabetic ß-thalassemia patients: Hepatitis C and deferasirox.

BACKGROUND AND AIM: Hypozincemia is a prevalent adverse consequence in diabetes mellitus (DM) and ß-Thalassemia patients. We aimed to evaluate the level of serum zinc in ß-thalassemia patients with DM and a risk assessment for hypozincemia. METHODS: The study population included transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT) with overt DM (fasting plasma glucose (FPG) ≥126 mg/dL, and/or 2-h plasma glucose≥200 mg/dL). Serum zinc concentration was measured by the colorimetric method, and the values below 70 µg/dL were defined as hypozincemia. Myocardial and liver T2*-weighted magnetic resonance imaging (MRI T2*, millisecond [ms]) were valued by a free contrast MRI. The demographic, clinical, paraclinical, and laboratory data were also recorded. The data belonged to the period from December 2018 until December 2020. RESULTS: Of 64 diabetic ß-thalassemia patients, 41 cases had zinc data in their medical files (aged 38 ± 9 years, 48.8% female). 78.05% of patients (n = 32) were TDT, and 21.95% were NTDT (n = 9). The mean ± standard deviation of zinc level was 110.2 ± 127.6 µg/dL. The prevalence of hypozincemia was 9.76%, 95% confidence interval [CI] 0.27 to 19.24 (four cases). After controlling age, the odds of hypozincemia for using deferasirox (DFX) was 8.77, 95% CI 0.60 to 127.1. In ß-thalassemia patients, the age-adjusted risk of hypozincemia was calculated at 15.85, 95% CI 0.47 to 529.3 for hepatitis C. The adjusted risk of hypozincemia based on age for antacid use was 6.34, 95% CI 0.39 to 102.7. CONCLUSION: In light of this study, as well as hepatitis C, using DFX and antacids is associated with a high risk of hypozincemia amid diabetic ß-thalassemia cases. However, upward bias should be taken into consideration.

Congenital duodenal obstruction with delayed presentation: seven years of experience.

INTRODUCTION: The duodenum is the most common site for congenital intestinal obstruction. The duodenal web with a central hole can present without any overt signs of obstruction at a later age. MATERIAL AND METHODS: Over a 7-year period, children with congenital intestinal obstruction were identified in this study. The complications and operative findings of patients with duodenal web with conditions such as wind sock deformity and delayed diagnosis were evaluated in this study. RESULTS: This study included 81 infants with congenital intestinal obstruction. At operation, 48 patients demonstrated duodenal obstruction with atresia in 27, annular pancreas in 15 and malrotation in 6. Also, we observed incomplete obstruction of the duodenum due to a fenestrated web in 8 patients. The age of these patients at operation time ranged from 5 days to 72 months. Fifty percent of affected patients were associated with trisomy 21, all of whom exhibited failure to thrive due to food intolerance. The patients older than 2 years presented with major complications of gastroesophageal reflux such as esophageal ulcer, stricture and dysphagia. Additionally we had a unique case of a patient who had been referred with an epigastric mass. During the operation, we found 440 seeds of various fruits in the stomach and the first portion of the duodenum. CONCLUSIONS: Our study emphasizes that duodenal web with a central hole can present without signs of obstruction at a later age with only failure to thrive and food intolerance. Therefore a high index of suspicion is necessary for diagnosis when it presents beyond the usual age.

Health-related quality of life (HRQOL) in children with chronic liver disease in North East Iran using PedsQL™ 4.0.

BACKGROUND: Health-related quality of life (HRQOL) is a concept that relates to an individual's perception of health status in relation to the culture and value systems in which they live, in addition to their expectations, goals, concerns, and living standards. Considering the size of the population affected by Chronic Liver Diseases (CLDs) and the severity and chronic nature of the symptoms, there is an emerging need to evaluate the quality of life of patients using a standard protocol. The aim of this study is to assess the HRQOL in children with CLD based on child self-report and parent proxy-report forms. METHODS: A total of 164 children, 55 CLD and 109 healthy children (aged 6-17 years), upon referral from the Pediatric Department at Ghaem Hospital in Mashhad from 2010 to 2014 were enrolled in this case-control study. We used the PedsQL(TM) 4.0 generic score scale to assess the HRQOL in children with CLD compared to the control group based on child self- and parent proxy reports. RESULTS: According to the child self-reports, the total HRQOL in the case group (89.93±9.63) was significantly lower than control group (93.05±9.28) (p=0.006). We found significant differences in emotional functioning based on the CLD child self-reports (p=0.001) and their parent proxy-reports (p=0.002). Furthermore, there was a statistically significant correlation between the severity and physical functioning as reported by the Child-Pugh score (p=0.03, r= -0.31) and the MELD/PELD scores (p=0.01, r= -0.35), based on child self-reports. Gender, age of onset, CLD types, duration of the disease, and treatment showed no significant differences with total HRQOL. CONCLUSION: HRQOL is significantly lower in children with CLD in comparison to the normal population. We strongly recommend considering different aspects of quality of life, especially emotional functioning concomitant to the therapy programs.

The effect of extracorporeal shock wave lithotripsy in the management of idiopathic gallstones in children.

OBJECTIVE: The most common etiology for gallstones in children is hemolytic diseases; however, the prevalence of nonhemolytic gallstones, which are mostly idiopathic, is increasing. Several studies concerning the treatment of gallstones with respect to the influence of extracorporeal shock wave lithotripsy (ESWL) have been conducted in adults, but not to the same extent in children. Therefore, this study attempted to examine the effects of lithotripsy on idiopathic gallstones in children. MATERIALS AND METHODS: In this study, 12 children, all of whom were under 12 years of age and diagnosed with idiopathic gallstones, were treated with ESWL. The average age of the children examined in this study was 6.5 years (range 3-11 years). Patients were treated with 2500-3000 shockwaves per session. The number of shockwaves was 90 shocks/min and the impulse intensity ranged from 10 to 12 kV. The final goal was the fragmentation of stones in pieces with less than 3 mm in dimension. Patients were followed up for 6-30 months. RESULTS: A total of 12 patients were treated with ESWL for 14 rounds. In three patients, complete fragmentation occurred within the first trial and was cleared. The nine remaining patients underwent ESWL 10 times in which an acceptable change in the gallstone's condition was not observed. Five of the patients underwent surgery. The chemical composition of the gallstones showed that the dominant element in them was calcium. CONCLUSIONS: Our findings show that performing ESWL can be effective in some children. Further studies with larger population are recommended. Furthermore, it seems increasing the voltage intensity and frequency as conducted in adults accompanied with biliary acids prescription can be effective in children.

Intractable chronic constipation in children: outcome after anorectal myectomy.

BACKGROUND: Many children with constipation fail to respond with conventional medical therapy. Surgery can produce a good result in dysfunction of the colon secondary to aganglionosis. However, its role in treating idiopathic constipation is more controversial. PATIENTS AND METHODS: A consecutive series of 44 patients with chronic idiopathic intractable constipation were included in this study. All children were investigated by barium enema and anorectal manometry. Due to inadequate response to medical therapy, all of these patients were selected for internal sphincter myomectomy. Patients were followed-up from 3 to 12 months. RESULTS: Short-term (3 months) and long-term (6 months) follow-up was available for all patients. The histology examinations showed normal ganglion cells in 32, hypoganglionosis in eight and aganglionosis in four patients. In short-term, regular bowel habits, without the need for laxatives or low dose drugs were recorded in 35 patients (79.5%). Overall there was an improvement in 68.2% of the children after 6 months follow-up. There was not any correlation between histopathological findings, duration of symptoms, age and sex of operation and response to myectomy. CONCLUSION: anorectal myectomy is an effective procedure in patients with intractable idiopathic constipation. It relieves symptoms in 68.2% of patients with chronic refractory constipation.

Partial internal biliary diversion in progressive familial intrahepatic cholestasis: introduction of a new approach.

INTRODUCTION: Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma. CASE PRESENTATION: An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostomy with myotomy operation. Because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and intussuscepted valve surgery. She was followed for 9 months. Despite disappointing outcomes of internal drainage with cholecystoappendicostomy, results of cholecystojejunocolic anastomosis with intussuscepted valve surgery were promising. DISCUSSION: The cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis.

Hyaline vascular-type Castleman's disease in the hilum of liver: a case report.

BACKGROUND: Castleman's disease or angiofollicular lymphoid hyperplasia is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. The disease can present at any extra thoracic site with lymphoid tissue such as retroperitoneal, mesentery, axilla, and pelvis. Hepatic localization castleman disease is very rare in children. Herein, we reported a case of Castleman's disease arising from the lymph node in hilum of liver. CASE PRESENTATION: A 5 -year-old girl with chief complaint of abdominal pain for two months which exaggerated in last three days was referred to the hospital. On routine physical examination, only a generalized abdominal pain was noticed. Routine laboratory investigations and Chest X-Ray were normal. Abdominal Sonography revealed a 3.7 x 3.1 cm solid mass in the hilum of the liver. On the MRI images, a lobulated mass in the portal hepatic associated with mass effect on the portal vein was visible. Histological examination revealed expansion of mantle zone in lymphatic nodules accompanied by burnt out germinal centers. This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease. The patient underwent a laparotomy. The patient had an uneventful postoperative course. CONCLUSION: This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease.