Osman Z. Karakuş

Effect of Meconium on the Contractility of the Superior Mesenteric Artery: A Clue to Intestinal Damage in Gastroschisis

INTRODUCTION Intestinal damage has been shown to occur when intra-amniotic meconium concentration exceeds threshold level. However, the mechanism of the meconium-induced intestinal damage is still unclear. Intestinal ischemia can cause intestinal damage in gastroschisis. This study was aimed to determine the effects of intra-amniotic meconium on the contractility of superior mesenteric artery (SMA). MATERIALS AND METHODS Eighteen-day-old fertilized chick embryos (Gallus Domesticus) were extirpated and intestines were harvested. The SMA specimens were prepared as 4 mm segments in the organ bath with Krebs-Henseleit buffer. The isometric contraction responses of the SMA specimens were evaluated with norepinephrine, different meconium concentrations, and clear amniotic fluid. Maximum isometric contractions responses (MICR) of the SMA specimens were recorded with an amplifier system on a computer. RESULTS In the norepinephrine group, MICR was found as 2.92 ± 0.57 mN. While MICR of the 1/100 meconium group (highest meconium concentration) was found as 1.56 ± 0.40 mN, MICR of the clear amniotic fluid group was 0.41 ± 0.07 mN. The MICR of the norepinephrine group was significantly increased compared with the 1/100 meconium and clear amniotic fluid groups. MICR of the 1/100 meconium group was also found to be significantly increased compared with clear amniotic fluid group. No statistically significant difference was found among the meconium subgroups. CONCLUSION Intra-amniotic meconium in fetuses with gastroschisis might cause ischemic intestinal damage by reducing the intestinal blood flow. Further studies are needed to show the outcomes of the vasoactive effect of meconium on the SMA blood flow.

Pectus carinatum: the effects of orthotic bracing on pulmonary function and gradual compression on patient compliance

OBJECTIVES The treatment of pectus carinatum (PC) deformity has been considered to be operative. Some authors have shown that postoperative pulmonary function is worsened. They have suggested that compromised chest wall expansion secondary to surgery leads to compromised pulmonary function. Several authors have advocated an orthotic brace for the treatment of PC. Pulmonary functions after orthotic brace treatment have not been investigated. METHODS Between April 2006 and October 2012, 61 patients presented with PC. Orthotic braces allowing gradual compression were prepared according to the anthropometric measurements of individual patients. The brace belt was tightened gradually. The brace was worn 6 h a day during the first week and the bracing time was prolonged for an additional hour per week till 16 h per day has been reached. Pre- and post-treatment echocardiography, pulmonary function tests and thorax computed tomography (CT) were obtained. The pectus severity index (Haller index) and the angle of sternal rotation were measured using CT. Satisfaction from bracing was evaluated by parents or patients at the end of the treatment. RESULTS While the mean pretreatment Haller index was 1.96 ± 0.24, the mean post-treatment index was 2.26 ± 0.32. The angle of rotation was improved by 47.5%. Forced vital capacity and forced expiratory volume in 1 second were correlated with the predicted values for age. There was no statistically significant difference between pre- and post-treatment values. No skin breakdown or bruising was encountered. The overall average satisfaction score was 3.92 ± 0.27. CONCLUSIONS We conclude that pulmonary function tests are not affected after brace treatment and gradual progression of bracing increases the patients compliance.

Tubularized incised plate urethroplasty for the treatment of penile fistulas after hypospadias repair

OBJECTIVE Urethrocutaneous fistula is the most common complication of hypospadias repair. Tubularized incised plate urethroplasty (TIPU) has been used for the management of distal fistulas. This study reports the usage of TIPU in the treatment of large penile fistulas. MATERIALS AND METHODS Between April 2002 and September 2012, 15 patients with large penile fistulas who were managed with TIPU were included in the study. The fistulas were sited along the penile shaft from proximal to distal penile localization. Glanular and coronal fistulas were excluded. The surgical technique was completed according to the standard TIPU technique. The surrounding scar tissue of the fistula was circumferentially excised, and the urethral plate at the level of the fistula was incised to provide performance of loose urethral tubularization. A urethral stent was kept for 5-7 days. RESULTS The mean age of the patients was 7.3 ± 3.1 years. Primary operation of these patients was tubularized preputial island flap (n = 6), on-lay preputial island flap (n = 4), and TIPU (n = 5). The sites of the hypospadias fistulas were as follows; penoscrotal (three), mid-penile (eight) and subcoronal (four). Fistulas recurred in two patients after fistula repair. The postoperative follow up of the patients was 12.4 ± 7.7 months. CONCLUSION TIPU may be used safely for the treatment of fistulas after hypospadias repair.

The intestinal damage induced by lipid soluble meconium subfraction is profound compared to the intestinal damage induced by water soluble meconium subfraction

BACKGROUND It is unclear which substances in meconium are responsible for intestinal damage in gastroschisis. An experimental study was designed to investigate the effects of the lipid or water soluble subfractions of meconium on the intestines of gastroschisis in a chick model. METHODS Meconium was pooled, homogenated, rota-evaporated dry and diluted. Meconium subfractions were obtained from water soluble and lipid soluble extracts of the meconium. Five days old fertilized chick embryos were used and divided into 5 groups: control, sham, water soluble meconium subfraction, lipid soluble meconium subfraction and whole meconium. All embryos were extirpated on the 18days and the intestines were harvested for histopathological examination. Serosal thickness was measured under light microscopy. RESULTS Serosal thickness of the meconium (36.36±2.8μm), the water soluble meconium (14.15±0.93μm) and the lipid soluble meconium (23.88±1.69μm) subfractions groups were significantly increased compared with the control (7.47±0.68μm) and the sham (7.48±0.71μm) groups (p<0.001). Serosal thickness of the lipid soluble meconium subfraction group was significantly increased compared with the water soluble meconium subfraction group (p<0.001). Serosal thickness of the meconium group was significantly increased compared to both the water and the lipid soluble meconium subfraction groups (p<0.001). CONCLUSION Lipid soluble meconium subfraction induces more intestinal damage compared to water soluble meconium subfraction.

Nuss procedure: Technical modifications to ease bending of the support bar and lateral stabilizer placement

Background: Modifications defined to ease bending of the support bar and lateral stabilizer placement during minimal invasive repair of pectus excavatum (MIRPE) have not been reported. We herein report our experience with MIRPE including several technical modifications. Methods: A total of 87 patients who underwent MIRPE were evaluated retrospectively. Technical modifications are (1) a template drawn preoperatively according to the anthropometric measurements, (2) more laterally placed thoracal incisions, (3) single existing incision for multiple support bars, (4) to secure lateral stabilizers to support bar in inverted position. Results: The mean patient age was 11.2 ± 3.8 years. The mean operating time was 63.7 ± 18.7 min. The mean Haller index was 5.4 ± 2.1. Eight patients necessitated two support bars. The support bars were removed in 69 patients after the completion of treatment. Support bars were left in place 26.8 ± 4.3 months. Final chest contours of the 56 patients were evaluated as 12 months passed after support bar removal and excellent repair results were determined in 84.2%. Conclusion: Preoperative bending of the support bar according to anthropometric measurements and fixation of the lateral stabilizers to the support bar in inverted position facilitates bar shaping and lateral stabilizer placement.

Intra-amniotic administration of urinary trypsin inhibitor preserves intestinal contractility in meconium induced intestinal damage in chick embryos with gastroschisis

BACKGROUND Intestinal damage causes intestinal dysmotility in gastroschisis. Urinary trypsin inhibitor (UTI) has been shown to prevent intestinal damage in chick embryos with gastroschisis. The effect of intra-amniotic administration of UTI on intestinal motility in gastroschisis has not been investigated. METHODS Five-day-old fertilized chick embryos were used. Gastroschisis was created through the amniotic cavity without opening the allantoic cavity. There were six groups; control, gastroschisis only, gastroschisis plus meconium and three treatment groups. In the treatment groups, 100 IU/mL, 200 IU/mL and 400 IU/mL UTI were instilled into the amniotic cavity of the gastroschisis plus meconium embryos, respectively. Serosal thickness of the intestines in each group was measured histopathologically. The contractions of the intestines were evaluated by in vitro organ bath technique and the responses were expressed as maximal contraction induced by acetylcholine. RESULTS The serosal thickness was significantly increased in the gastroschisis plus meconium, 100 IU/mL, 200 IU/mL UTI groups compared to control and gastroschisis only groups. The serosal thickness of the 400 IU/mL UTI group was similar to control and gastroschisis only groups. Contractility of the intestines was diminished in the gastroschisis plus meconium, 100 IU/mL and 200 IU/mL UTI groups. There was no significant difference regarding contractility among control, gastroschisis only and 400 IU/mL UTI groups. CONCLUSION Intra-amniotic administration of UTI preserves intestinal contractility in chick embryos with gastroschisis. However, preservation of intestinal dysmotility by using UTI in the human gastroschisis cases needs further experimental and clinical trials.

Child with ret proto-oncogene codon 634 mutation

İnce D, Demirağ B, Ataseven E, Oymak Y, Tuhan H, Karakuş OZ, Hazan F, Abacı A, Özer E, Mutafoglu K, Olgun N. Child with RET proto-oncogene codon 634 mutation. Turk J Pediatr 2017; 59: 590-593. Herein we reported a 7-year-old child with RET proto-oncogene c634 mutation. Her mother had been diagnosed with medullary thyroid carcinoma (MTC), and treated six years ago. Heterozygous mutation of the RET proto-oncogene at c634 had been detected in her mother. Genetic analysis showed the presence of the same mutation in our patient. Thyroid functions were normal. Serum calcitonin level was found mildly elevated. Parathormone (PTH) and carcinoembrionic antigen (CEA) levels were normal. Prophylactic thyroidectomy and sampling of cervical lymph nodes were performed. Histopathologic examination revealed hyperplasia in thyroid C cells, and reactive lymphadenopathy. The risk of MTC has been reported 100% through the life of patients with RET proto-oncogene mutation. It has been reported that particularly patients with c634 mutation have more risk of occurence of metastatic and progressive/recurrent MTC. Prophylactic `thyroidectomy, cervical lymph node dissection` before 5-years-of-age should be considered for these patients.

The role of water-soluble meconium subfraction and lipid-soluble meconium subfraction on the superior mesenteric artery vasoconstriction in chick embryos

OBJECTIVE Intraamniotic meconium has been responsible for intestinal damage in gastroschisis and meconium-dependent intestinal ischemia has been proposed to induce additional intestinal damage in gastroschisis. This study is aimed to determine the effects of lipid and water-soluble meconium subfractions on the contractility of the superior mesenteric artery (SMA). MATERIALS AND METHODS The study was conducted on 18-day fertilized chick embryos (Gallus Domesticus). Meconium is fractioned into water and lipid-soluble components. Only one SMA tissue was prepared from each embryo and suspended in the organ bath. Isometric contraction responses (ICR) were created in SMA tissues by one hour of incubation in Krebs-Henseleit solution for each group. Groups consisted of control, meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction. ICR of the SMA specimens were evaluated with a transducer-amplifier system on a computer. The data were expressed (mean±1SD) as milliNewton (mN). RESULTS The ICR of the meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction groups were significantly high when compared to the control group (p<0.01). The meconium and water-soluble meconium subfraction created more contraction response than the lipid-soluble meconium subfraction (p<0.01). The ICR of the meconium group was not different from the ICR of the water-soluble meconium subfraction group (p>0.05). CONCLUSION Water-soluble meconium subfraction has a profound vasoconstrictor effect on the SMA compared to the lipid-soluble meconium subfraction.