1st International Medical Conference: Advances in Pediatric Care

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

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.

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.

The recognition of the congenital anomalies involving the base of the skull CCJ region is important because of their association with brain stem-spinal cord compression, spinal instability and spinal deformity. Other organ system anomalies may be associated with CCJAs as these systems share common embryonic development. A basilar impression with neural compression in a form of bulbar and cervical myelopathy with associated occipito-cervical rotational instability levelled to occipito-axial hypoplastic occipitalised atlas caudally, to brain stem medullar neural compression bulbar ward, was diagnosed in a 14 year old boy who was admitted and managed at MIH-Jerusalem.

Lead and it's compounds are used as additives to several products such as gasoline and paints. Lead has a toxic effect especially on brain and nervous system. Almost no published work has been found providing information about blood lead levels in children of Palestine. And thus the present work was carried out .An improved (ASV/HDME) method for determination of lead in whole blood by anodic strippingvoltametry (ASV) using hanging dropped mercary electrode "HDME" has been developed with a special reagent at An-Najah N, University laboratory. A total of 518 sample from 10th grade students of Jenin district, the samples have been analyzed using the mentioned improved method>The geometric mean of blood levels was 87.75 μg/L, a variation in the geometric mean of blood lead level was noticed with respect to students place of residence;( Camp student 119.43 μg/L), (City students 92.41μg/L), ( Yammon village students 77.65 μg/L). also a variation appeared with respect to27 First International Faculty of Medicine Conference 2008school location, and the highest mean 185.71 μg/L was found in Al-Sallam males school which located near a heavy traffic movement and near an industrial area. In each sample statistic was carried out to seif there was any correlation between blood lead levels and lead risk factors. Factor that found to affect blood lead levels are; gasoline stations, paints workshops, highway' incinerators and quarries. Kidney colic joint pain, R. T. disorder and C. V. disorder appears with increasing blood lead level. Also blood lead level was higher in persons using tea and flour from local mills, and increased in persoms with lower parents education and income level.

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.

Introduction: We reported our experience of long bones fixation in 16 children with Osteogenesis Imperfect a by Nancy Nails over the course of five years. In the past two years we have performed fixation of long bones of lower limbs in 4 children with Osteogenesis Imperfect a using Fassier-Duval telescopic nail.

Patients & Methods: The average age of patients in the group treated by telescopic nails was 7.1 (5-8) years. All four children were diagnosed as Osteogenesis Imperfect a, two with Type I, one with Type III and one had an unrecognized type. All patients had an anamnesis of at least 8 previous pathologic fractures of limbs; in two of them pathologic vertebral fractures were also found. Two of the patients had previous surgery and underwent fixation by Nancy nails, the result of which was considered a failure. One patient had Coventry plating of hip fracture one year prior to telescopic nailing. The same patient had developed severe bilateral genu valgum which was treated by 8-plate patial epiphyseodesis one-half year before nailing. Six intramedullary telescopic femoral nailings and 4 intramedullary tibial nailings (10 procedures) were performed in four patients. In all patients multiple osteotomies of bone were performed in order to correct severe deformity. Soft bandage (Johns) was applied after surgery. Weight bearing was allowed after appearance of callus on control X-Ray (8-14 weeks after surgery, depending on age and weight of the child). All children received chronically treatment by infusions of Pamidronate.

Results: Fracture healing at osteotomy site was achieved in all cases. All patients were free of pain. No cases of infection were observed, nor were cases of postoperative contracture of knee joint. No growth arrest was observed after surgery. Normal alignment was restored in all cases with previous malalignment of femur and tibia. Two of patients in this series were not walkers and did not begin to walk after the surgery. One patient with OI Type III perforation of distal locking of “male” nail through femoral condyle into knee joint was re-operated and the distal part of the nail was secured into the distal femoral epiphysis by bone cement.

Conclusions: We consider fixation by Nancy Nails as inappropriate for treatment of Osteogenesis imperfect a. Fassier-Duval telescopic nail has proven useful for fixation of long bones in Osteogenesis Imperfect a and more secure than other devices used before for treatment of this pathology. The surgical technique is complicated but well developed.

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

Skin diseases are common in general practice in Palestine but there is no data available about that. In a study by Dr.Adnan Kamal and H.Arda on 23583 Dermatology patients seen 1972 – 1979 the infective group formed 36 % , Dermatophytes 12 % and Tinea capitis ( T.C. ) 4.2 % .In a study by H.Arda 1978 at primary schools in some villages , Refugee camps and the city of Nablus for the diagnosis and treatment of T.C. which showed an incidence of 15 % , 3 % and 2 %respectively . All patients were treated and the same schools were seen on two occasions 1979 and 1996to see that T.C. was dramatically reduced .A mass campaign treatment program was carried out 1983 – 85 , in which a total of 101852 schoolchildren below the age of 14 were examined all over the West Bank for T.C. , of them 3347 children were found to have T.C. ( 3.3 % ) . In Jenin Governorate the highest rate of infection was found 7.2 %and in Jericho the lowest 0.6 % .Recently we looked for skin infections and infestations between all Dermatology patients seen during2000 – 2007 with results showing high frequency of all these conditions in children .In the treatment of T.C. we are using griseofulvin F.P. in a dose of 10 – 15 mg / Kg weight for 6 – 8weeks with very good response and rare complications. In scabies we use lindaine and permethrin as one application for 8 hours and all members of household should be treated at the same time. In Impetigo we insist always at the use of systemic and local antibiotics for rapid relief of symptoms. In warts we use treatment according to the site of lesion and age of the patient.

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.

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.

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.

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.