1st International Medical Conference: Advances in Pediatric Care

The adriamycin-induced rat model of the Vertebral, Anorectal. Tracheo-Esophageal. Radial and Renal (VATER) association produces a variety of vertebral, rib, and limb abnormalities. This study was designed to document accurately the nature of these abnormalities and to determine whether synovial joints are affected. Fetuses from pregnant Sprague Dawley rats that had received intraperitonealinjections of 1.75 mg/kg of Adriamycin on days 6-9 or 10-13 of gestation were harvested. Doublestainedskeletal preparation and histological sections were examined for vertebral, rib, and limb anomalies. The incidence of abnormalities was high in the group treated on gestational days (GD) 6-9,while it was low in the GD 10-13 group. The length and thickness of the long bones were reduced, with bowing and reduction in their endochondral ossification. Sirenomelia occurred in the group treated onGD 6-9, and was often associated with a short tail and anal atresia. The joint cavities and intra-articularstructures such as menisci and the cruciate ligaments developed normally from the mesenchymalinterzone. These data indicate that Adriamycin inhibits skeletal growth and differentiation without any interference in the differentiation of the mesenchymal interzone, thus producing normal synovial joints.

Patients with thalassemia major who receive regular blood transfusions are likely to develop iron overload. This will result following saturation of the iron carrying capacity of transferring, which generally takes place after 20 transfusions. Consequently, excess plasma iron (labile iron) is cleared rapidly by the liver, heart and endocrine tissues at a rate that exceeds 200 times normal uptake of transferring-bound iron. Excess labile iron within cells will destroy the structure and function of mitochondria, lysosomes, lipid membranes, proteins and DNA. The clinical consequences can include liver cirrhosis and fibroses, cardiomyopathy, diabetes and other endocrine disorders. With proper control of body iron at all times in patients, these effects are preventable but only some are reversible once tissue damage has occurred. The primary rate of iron chelating therapy is to bind and remove iron from the patient body at a rate either equal to or greater than the rate of iron uptake of transfused iron. Complete chelating should aim to achieve both iron balance and iron detoxification. In patients who accumulated dangerous tissue iron levels, removal of this iron is also highly desirable. Several iron cheaters have been developed to help achieve these objectives. The recent introduction of the effective oral cheater deferasirox provideschelation coverage and significant control of LPI levels over the entire 24 hour period. Evidently, administration of the drug at a dose of 20-30 mg/kg/day significantly controls iron levels in plasma, liver and myocrdiocytes in thalassemi a major patients. Accumulating clinical data from key studies indicate that thalassemia patients can lead a happy and enjoyable normal life similar to normal individuals if their treatment is performed right and they receive the proper clinical attention from the medical staff. Definitely, chelating therapy requires close monitoring of patients vital organ status and the treatment protocol should be adjusted accordingly. Long term treatment with iron chelation highlights the importance of titrating the dose of the drug for each patient according to individual rates of iron intake from continued blood transfusion, current iron storage levels, safety markers (renal function for example) and target body iron content desired.26 First International Faculty of Medicine Conference 2008The presentation will highlight some of the recent clinical data from leading clinicians In chelationtherapy from several parts of the world and the lessons that can be learned from their experience and work for the benefit to optimize the treatment of thalassemia major patients in our population.

Introduction: Despite the widespread use of screening programs to detect hip dysplasia in the
newborn, children are still seen later in childhood with established dislocation. We retrospectively evaluated the radiographs of 59 cases of open reduction, with and without Salter osteotomy, both to evaluate the long-term effects of the Salter osteotomy on the quality of the hips and to decide if it should be a routine adjunct to open reduction. We also compared the hips with the Salter osteotomy done at the time of primary open reduction with those in which it was done later as a secondary procedure to determine if the procedure actually affects the development and modeling of the acetabulum. This series is distinguished by the long follow-up to, or almost to, skeletal maturity.

Methods: We reviewed the records and x-rays of sixty two (59) hips with DDH with no associated congenital or neuromuscular disorders who underwent open reduction as primary surgical treatment after the age of 1.5 years. All patients had their primary treatment in our hospital between 1975 and 1992 with a minimum follow-up of 9.6 years. There were 36 hips that had only open reduction as a primary procedure (Group R) and 26 hips that also had a Salter osteotomy (Group RS). In Group R there were 4 hips that had a subsequent secondary Salter osteotomy (Group R/S, the slash representing an interval of time). 15 First International Faculty ofMedicine Conference 2008 We evaluated radiographs done preoperatively, postoperatively and at the time of the most recent follow up visit. We measured the acetabular index (AI), center edge (CE) angles, and the sphericity of the femoral head by the Mose technique. The hips were assessed for avascular necrosis by the method of Kalamchi and MacEwen and the overall outcome graded according to Severin.

Results: For Group R and Group RS, the mean ages at first operation were 4.9 years and 3.6 years respectively, and the mean radiological follow-ups were 11.3 years and 9.6 years. The pre-operative acetabular indices were 35.0º and 35.2º indicating that the severities of dysplasia were similar. 57.5% of Group R went on to have subsequent surgery compared to 23% of group RS. In groups R & RS respectively, assessment of outcome at final follow-up revealed CE angles of 26.5º and 30.1º, mean Mose circle indices of 3.8º and 2.4º, avascular necrosis presence in 57.5% and 31%, and good (grades 1 & 2) Severin results in 63% and 81%. In groups R/S & RS the pre-operative acetabular indices were 31º and 37.6º indicating that the hips in group R/S were less dysplastic to start. Assessment of outcome at final follow-up revealed, in groups R/S and RS respectively, CE angles of 34.0º and 29.8º, mean Mose circle indices of 2.5º and 2.1º, avascular necrosis presence in 50% and 31%, and good (grades 1 & 2) Severin results in 75% and 85%.

Conclusions: Our results show that routinely performing a Salter osteotomy at the time of primary open reduction of the hip improves the long-term outcome of the hip. The femoral head is a better shape as evaluated by both the Mose and Severin methods and the acetabulum provides better coverage as indicated by the center-edge angle. In addition, fewer subsequent surgical procedures were required and evidence of avascular necrosis was less.
It might be argued that the only effect of the Salter osteotomy is a fixed geometrical change, that it should not matter in the long run whether that change is introduced early or later, and that the Salter osteotomy could be omitted from the primary procedure and performed later only for persistent dysplasia. Having only 4 cases in our R/S group prevents us from reaching reliable conclusions but the fact that group RS was more dysplastic at the start, and arguably better at the end suggests that there is an advantage in doing the Salter osteotomy early.

Significance: These results should cause those surgeons who do not routinely include the Salter osteotomy in the primary surgical treatment of the dislocated hip to reconsider their strategy in terms of including the Salter osteotomy in their primary surgical treatment of the dislocated hip over 1.5 years of age.

Purpose: Perineal & scrotal forms are the most severe, uncommon and challenging variants in the spectrum of hypospadias These forms are often associated with extreme chordee, penoscrotaltransposition and bifid or absent scrotum We reviewed cosmetic and functional outcome of masculinizing genitoplasty (MGP) in intersex patients assigned as a male performed in our service in the last 6 years.

Materials and Methods: A total of55 patients underwent MPG by one surgeon during 6 year period. Patient age ranged from 6 months to 16 years .The presenting disease was severe hypospadias or intersex, scrotal hypospadias in 30 cases , and perineal hypospadias in 25 .Some patients were evaluated for gender, chromosomal and biochemical data by pediatric endocrinologists. Most of our patients had idiopathic male pseudohermaphroditism.. MGP was performed using Augmented Koyanagi Nonomura one-stage surgical techniques in the first 2 years,then we used extended unilateral parameatal flape with minimal mobilization technique in the next 4 years, dorsal plication needed in7 patients.Scrotoplasty and orchiopexy was performed in the same operation. 21 First International Faculty of Medicine Conference 2008

Results: Followup was 1 to 6 years. Satisfactory cosmetic and functional results in terms of good urinary stream and straight phallus in ( 72%) of cases . Complications requiring reoperation occurred in (28%). complications presented are various degrees of failure of the urethroplasty or cosmetic appearance.95% of patients have satisfactory result after the second operation.

Conclusions: Male pseudohermaphroditism better to be managed by single stage male genitoplasty, a second operation may be needed only in about 25% of cases ( patients with complications from the first operation) . In order to achieve a longer new urethra we recommend our modified technique – the extended parameatal-based flap with minimal moblization urethroplasty.

Type 1 diabetes is one of the most frequent chronic diseases in childhood. According to PalestinianMOH, there are 1342 registered patients following up for type I diabetes in the West Bank PHC’s,which makes 6.5% of all diabetics following up at these clinics; about 20% of those patients are from Hebron, and 18% from Jenin.The incidence rate (The number of new patients per100000 diagnosed as type I diabetes in these clinics for the year 2007) was 2.1M and 2.0 F for the age group 0-4,9.7M,7.3F for the age group 5-14. Its noticeable that incidence increases with age and is higher in males than females.28 First International Faculty of Medicine Conference 2008In Type 1 diabetes, lack of adherence to treatment and a failure to maintain glycaemic control can lead to long-term complications& loss of quality of life. Young age Children are at higher risk of longtermcomplications. Chronic illness places the adolescent at a greater risk because of added emotional difficulties and stress in coping with rigorous regimens and restrictions. Other problems are economic and social, in the form of stigma like delay in marriage or reduced job opportunities. Among the several problems faced by the young diabetic, the most common and often most difficult to deal with is that of adherence or compliance to a regimen. Children with diabetes in developing countries face profound challenges in managing their diabetes. Obstacles in Palestine are similar to universal ones mentioned. In my experience important obstacles in Palestine are; lack of cooperation & coordination between health providers, lack of national treatment guidelines, insufficient preventive and control policies, shortage of human and financial resources, lack of well trained health provider team and important specialists, lack of patient and family education about management and coping, and finally inadequate financial coverage for the management cost. Proper management of diabetic children in Palestine as a developing country with special political situation, face many obstacles at different levels (national, MOH programs, family& school)My experience and my recommendations in Palestine will concentrate on cooperation of physicians and nurses to help diabetic children and their families cope with their disease, improve their quality of life by proper management & reducing morbidities. This can be achieved by follow-up of this group of patients in Proposed specialized well equipped clinic by a well trained team (physicians, nurses, psychologist, educator, and dietician), increase the awareness of their families and teachers about this disease and how to deal in emergencies (education programs), recruitment activities during holidays(camps, trips, sports), and home visits for special cases.

Background: Nephrogenic diabetes insipidus (NDI) is a clinical disorder characterized by a urinary concentrating defect resulting from resistance of the collecting duct to the antidiuretic action of vasopressin (AVP). NDI is classified into hereditary and acquired causes. X-linked recessive NDI is caused by mutations in the gene encoding the V2 vasopressin receptor (V2R) and is the most frequent genetic cause of the inherited NDI. Here we describe a novel mutation in the AVPR2 gene in a Palestinian family with NDI.

Clinical Data: A male infant, born to a non consanguineous Palestinian family, presented in the neonatal period with failure to thrive, vomiting, irritability & fever. Blood sodium was high up to 170mmol/L, blood osmolality raised over 330mOsm/kg while urine osmolalit remained low between 45-135mOsm/kg, urine output was 7cc/kg/hr & positive family history of a brother diagnosed previously to have NDI suggesting X-linked inheritance of the disease.

Molecular Data: Sequencing the AVPR2 gene revealed a novel mutation (C82Y) in affected patients in exon 2 of the gene, predicting Cysteine to Tyrosine substitution at the 82 amino acid residue of the AVPR2 gene, while the mother being carrier for the mutation and healthy brother and father does not have the mutation.

Conclusion: We describe a novel mutation in the AVPR2 gene in a Palestinian family with NDI, allowing early diagnosis to prevent severe dehydration and complications in addition to genetic counseling.

Purpose: We retrospectively evaluated the results of one-stag feminizing genitoplasty in children with congenital adrenal hyperplasia (CAH).

Patients And Methods: Twenty nine patients with 46XX CVAH (congenital verilized adrenal hyperplasia )patients were referred following complete evaluation of gender, chromosomal and biochemical data by pediatric endocrinologists in 6 year period between 2001 and 2007- at European Gaza Hospitan –Gaza ; 28 patient( new cases 26 + Redo 2)were managed by one-stage feminizing genitoplasty (vaginoplasty-valvoplasty and clitroplasty ),age 3monthes to 12 years.We used the Passerini-Glazel technique, and in 5 patients we mobilize the urogenital sinus en bloc , one female patient underwent gender reversal and was raised as males.

Results: Follow up was 3 monthsto 6 years. Preoperative ultrasound plus or minus genitogram
and endoscopy provide the correct data regarding the vaginal and internal genital anatomy in all cases, Operating time ranged between 90 and 180 minutes, average hospitalization period was 4 to 9 days Complications consisted of intraoperative uretheral injury in 1 case wound infection in 2 and stenosis at the suture line in 2 patients -corrected by introitoplasty . All patients have had successful cosmetic results except one need redo clitroplasty for residual clitoromegalia.

Conclusion: Most patients with 46XX CVAH are preferably raised as females and require a feminizing genitoplasty . Preoperative investigations and surgical managment of every case on its own merits gives satisfactory primary results , some revision at puberty should be anticipated in some cases.

Ambiguous genitalia is a rare disease but noted to be a common presentation in our pediatric endocrinology and urology clinics. The aim of our study is to identify the size of the problem, and to evaluate the family response to gender assignment.

Method: We review the files of the patients with ambiguous genitalia during a period of 5 years. A total of 80 children with ambiguous genitalia have been evaluated and treated. The three most common causes were Congenital adrenal hyperplasia CAH, deficiency of Ketosterod Reductase 17KR,and , deficiency of 5 alpha-reductase.. Most of the cases (42%) were from the north governorate .Consanguinity was very high (84%).17KR deficiency almost confined to the north governorate which indicates inbred gene effect in this area. Gender assignment was a problem in few cases, Social shame was present in almost all cases .The result of Surgical Reconstruction Gentiplasty of these Ambiguous genitalia was comparable to the international figures. 20 First International Faculty of Medicine Conference 2008

Conclusion: Though Gaza strip is a small geographic area there is marked geographic distribution differences of the cases which reflect the general attitude of the population towards consangeuous marriage and inbred gene effect. In general the early the diagnosis the easier to make decision on gender assignment to rear the child in a socially a acceptable role .Education of the medical personnel as well as the public education is highly recommended to reduce the impact of this health problem.

An intussusception is the enfolding of one segment of the intestine within another. An infant presented with Abd. Pain, constipation , and current jelly stool of 40 h duration. Barium enema failed to reduce the intussusception, thus laparatomy was done, milking of the intestine was failed and the seros started to rapture, thus a longitudinal incision was made in the ascending colon and by a finger an intralumenal reduction was done. Approximately 10% of affected children require surgical intervention, ether by squeezing the intestine to push the inner segment or by resection the gangrenous or not reducible intestine .This procedure is another option if there is difficulties in squeezing the intestine, but under certain conditions, 1- the bowel is healthy, 2- the intussusception is short segment.

Idiopathic hypertrophic pachymeningitis (IHP) is a rare but increasingly recognized disorder with localized or diffuse thickening of the dura mater of unknown etiology. The inflammation usually involves the cranial or spinal dura mater, with resultant neurological deficits. It has been primarily reported in adults, with focal involvement of the brain or spinal cord. We report a child who presented at the age of 3.5 years with IHP involving the whole neuraxis, with poor response to steroids,cyclophosphamide and intraventricular Cytarabine

Purpose: The aim of this retrospective study is to present all cases of congenital glaucoma seen in Gaza eye hospital in the period from 1997- 2008, geographic distribution, clinical forms, risk factors and results of therapeutic modalities

Methods: Data of all cases from their medical records including medical examination, clinical
evaluation at first presentation and surgical procedures involved in the management were evaluated.

Results: From this study we concluded that congenital glaucoma is one of the leading causes of blindness in children in Palestine with an incidence of 1: 3412 and high percentage of consanguinity. The surgical management in this study is similar to surgical management in other reports indicating that trabeculectomy with antimetabolites is the most common and rewarding procedure.

Objective: to provide an update to the original Surviving Sepsis Campaign clinical management
guidelines, "Surviving Sepsis Campaign Guidelines for Management of Severe Sepsis and septic shock," published in 2004.

Design: we used the grades of Recommendation , Assessment, Development and Evaluation
(GRADE) system to guide assessment of quality of evidence from high (A) to very low (D) and to determine the strength of recommendation. A strong recommendation:- Indicates that an intervention's desirable effects clearly outweigh its undesirable effects (risk, burden,
and cost) or clearly do not. Weak recommendations. Indicates that the tradeoff between desirable and undesirable effects is less clear. The grade of strong or weak is considered of greater clinical importance than a difference in later level of quality of evidence.
Recommendations are grouped into those directly targeting severe sepsis, recommendations targeting general care of the critically ill patient that are considered high priority in severe sepsis, and pediatric considerations.

Results: Recommendation specific to pediatric severe sepsis include greater use of physical examination therapeutic end points (2C); dopamine as the first drug of choice for hypotension (2C); steroids only in children with suspected or proven adrenal insufficiency (2C) and a recommendation against the use of recombinant activated protein C in children (1B).

Conclusions: There was strong agreement among a large cohort of international experts regarding many level 1 recommendations for the best current care of patients with severe sepsis. Evidenced- based 31 First International Faculty of Medicine Conference 2008 recommendations regarding the acute management of sepsis and septic shock are the first step toward improved outcomes for this important group of critically ill patients. (Crit Care Med 2008; 36:296-327)

Introduction: Clubfoot in a congenital deformity consisting of hind foot equinus, hind foot and forefoot varus and supination of the forefoot. In a complex deformity involving many bones, articulations and soft tissue structures. The deformity may be idiopathic or associated with congenital or neuromuscular problems. Plain x-ray is the most dependable radiological exam for diagnosis and prognosis of clubfeet. The ossific nucleus of navicular usually appears between 3 – 5 years of age. However in clubfeet the appearance of navicular might be delayed or when appearing it might look “abnormal in shape”. Here was retrospectively evaluated x-rays of 41 feet which were clubfeet who were treated surgically in Hospital for the first time by soft tissue release, and evaluating the relationship of the appearance of navicular with the prognosis of the surgery.

Methods: We reviewed the record and x-rays of 41 clubfeet with no associated congenital or
neuromuscular disorders who underwent soft tissue release as a primary surgical treatment. All the patients had their primary treatment in our Hospital between 1993 and 1998 with a follow-up of 5.9 years. There were 24 feet, which had normal shaped navicular and 17 feet with abnormal shaped navicular. We evaluated radiographs done pre-op, post-op and at the time of the most recent follow-up visit. We measured talocalcaneal angles, talohorizontal angles, space between articular surface of talus and cuniformis, length of navicular, level of dorsal subluxation of navicular.

Results: For the feet with avascularized navicular and the non-avascularized mean age at surgery was 1.0 year & 1.8 respectively. The mean radiological follow-up was 4.8, 6.9 respectively. Relapsed clubfoot was 15.7%, 16.6% respectively. Post-op infection 15.7, 8.3 respectively. Decreased distance between talus and cuniformis 36.8%, 20.8% respectively.

Conclusions: One result showed that avascular necrosis of navicular has no prognostic value in accordance with the complications of surgery. But increased incidence of avascular necrosis is related to the decreased distance between articular surface of talus and cuniformis and the age of the patient at time of surgery if the patient is less than 1 year old.

Significance: Avascular necrosis has no prognostic value in clubfoot post surgery but it is preferable when doing the surgery to do it in a child 1 year or above and not compressing the navicular between the talus and cuniformis during surgery.

Idiopathic hypertrophic pachymeningitis (IHP) is a rare but increasingly recognized disorder with localized or diffuse thickening of the dura mater of unknown etiology. The inflammation usually involves the cranial or spinal dura mater, with resultant neurological deficits. It has been primarily reported in adults, with focal involvement of the brain or spinal cord. We report a child who presented at the age of 3.5 years with IHP involving the whole neuraxis, with poor response to steroids,cyclophosphamide and intraventricular Cytarabine