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1. Transradial Angioplasty - Our Experience
  BK Goyal, Ramesh Kawar, BC Kalmath, Anil Sharma
 

Eighty patients underwent angioplasty by radial route over the period of one year in Bombay Hospital. Patients were randomly selected. Clinical presentation included stable angina, unstable angina, Acute myocardial infarction (MI), post MI angina and asymptomatic high risk group. 65 (81%) underwent angioplasty on adhoc basis and 15 (19%) were elective angioplasty.

No major complications at the entry site were noted in all the patients. Minor complications included radial artery spasm, pain at local site.

Procedural success was achieved in 76 (95%) of patients. All patients were ambulated immediately after the procedure.

Radial angioplasty is an elegant procedure, with high patient preference. No access related complications and high success rate.

 
 
1. Premature Ovarian Failure : A Clinical Profile
  Bhavesh Patel, VS Raut, AR Chauhan, Nalini Menon, AB Singhal
 

Background : Although prematurely failing gonads have been recognized since centuries, the mechanism involved in its failure and its oetiological classification have not been well understood.

Aims and Objectives : To study the various oetiological factors and their relative frequency. To evaluate the clinical, morphological, hormonal features of premature ovarian failure along with its various endocrinological associations. To develop a rational approach for evaluation and treatment.

Material and Method : The study was a retrospective analysis of 71 patients over a period of 6 years (1998-2003). All the patients were given postal intimation. 24 patients followed up for prospective evaluation.

Results : 71 cases of POF were analyzed over a period of 6 years. 30 (42.2%) patients were either Turner or Turner mosaic, 41 (57.8%) patients were non-Turner, causes of which were autoimmune in 20 cases (28.1%), idiopathic in 18 (25.3%), and pure gonadal dysgenesis in 3 (4.2%). Patients with Turner syndrome presented in second decade of life (mean age 16.1 yrs) with primary amenorrhoea (93.3%). Patients with other causes of POF presented in third and fourth decade of life (mean age 35.3 yrs) with secondary amenorrhoea. Of 71 patients 24 patients came for follow-up, of which only 13 patients were on treatment. Patients on HRT had 100% relief of menopausal symptoms and had well oestrogenised smears on vaginal cytology, as compared to those not on any treatment. Difference was statistically significant, and independent of the type and duration of treatment. Major cause of discontinuation of HRT was financial, making it the most important hurdle in effective management of the patient.

 
2. A Case Report of Broad Ligament Fibroid
  Rahul Salunkhe, Bhavesh Patel, AB Singhal
 

Although uterine fibroids lying in between two layers of broad ligament are common, but those arising from smooth muscle cells of round ligaments, utero-ovarian ligaments, and those covering uterine arteries (True Broad Ligament Fibroid) are relatively rare.

A 36 yr old patient, Mrs. XYZ P2L29 (FTND TL done), presented with chief complaints of lump in lower abdomen and minimal discomfort since last 3 months. She gave history of being operated for it in private nursing home 2-month back, however due to in situ findings of dense adhesions between bowels and broad ligament surgery was abandoned. On admission CT scan confirmed that the mass was of 12 cms and 10 cms in closed opposition to the left lateral wall of uterus and lying in broad ligament. Patient was subsequently explored after performing preoperative ureteric catherterization and surgeon standby. Broad ligament fibroid was excised and total abdominal hysterectomy was done. The postoperative course was uneventful and patient was discharged on day 7 of surgery.

The surgeon performing surgery for broad ligament should be well versed with the distortion in the normal anatomy of ureter and uterine artery. It would be prudent to delineate ureter either prior to surgery by IVP or CT scan; or it could be done intraoperatively via cystoscopic-guided ureteric catheterization. Irrespective of the modality used, after performing the procedure, ureter should be delineated in each and every patient.

 
3. An Unusual Case of Uterine Remnant
  Vanita Agawane, Bhavesh Patel, Pratima Chipalkatti, Rahul Salunke
 

Background : Although cases of Ovarian Remnant and Ovarian Residual Syndrome has been mentioned in literature, and are also seen often in clinical practice, a case of uterine remnant has not yet been recorded in literature till date.

Case History : A 29 year old patient, Mrs. XYZ, Para 3 Living 3, with history of vaginal hysterectomy done 8 months back for Fibroid uterus with menorrhagia, presented with chief complaints of cyclical monthly pain in lower abdomen since 7 months, and ultrasonography showing evidence of uterine remnant of 5 cms x 3 cms adherent to the superior surface of bladder. It is not infrequent to encounter a patient with history of 3 LSCS in modern day obstetrics for hysterectomy. CT Scan confirmed the diagnosis of uterine remnant on admission, and patient was explored. Postoperative course of the patient was uneventful and patient was discharged on day 5 of surgery.

Discussion : Although it would be prudent to presume that abdominal approach is better, but the final decision should rest with the doctor operating. If vaginal approach is contemplated, one should not hesitate to abandon the same for abdominal at any point in surgery, if difficulty thus arises. In such cases, adhesions are to be expected with both the bladder and the bowel, therefore it would be judicious to perform a pre operative CT Scan, and to ask for a Surgeon standby if the need thus arises. It would be also prudent to delineate the course of ureter, either prior to surgery by CT Scan, or intraoperatively by identifying and dissecting ureter or by cystoscopic-guided ureteric catheterization. Post operative integrity of the ureters should be confirmed in all cases.

 
4. Polyglandular Syndrome Type 1
  Deepak Pawar, Amit Tagare, Pankaj Kasar, Siddharth, Shilpa, Prem Sheth
 

Abstract : Polyglandular syndrome type 1 is one of the rare presentations in childhood. It is an autoimmune disorder, involving more than 2 endocrine glands and is characterized by mucocutaneous candidiasis, hypoparathyroidism and adrenal insufficiency.

Case Report : A 3 yr old female child 2nd in order of birth residing at Surat (Gujarat) referred to our hospital with multiple episodes of seizures since last 6 months.

On Examination : child was normothermic, vitals normal, skin coarse and dry, pallor +, angular cheilosis +, mucocutaneous candidiasis, no e/o rickets or neurocutaneous markers.

Eye exam normal, no cataract. Systemic examination NAD. Child investigated for s. calcium - 4.2 mg% (low), s. phosphorus - 10.6 (high), alkaline phosphatase - 128 (normal), s. electrolytes normal in view of low calcium pt started on iv calcium for 3 days f/b oral calcium. Pt still continued to convulse so endocrinologists opinion seeked i/v/o hypocalcaemic hyperphosphataemia with refractory seizures and advised to do thyroid and parathyroid hormone levels and CT brain. Upon investigation PTH - 1.2 pg/ml (low), TSH - 6.75 (high), CT scan brain : areas of calcification in b/l lentiform nuclei.

In view of a) clinical findings b) hypocalcaemic hyperphosphataemia c) low PTH, high TSH d) CT scan findings. Our diagnosis of polyglandular syndrome type 1 was confirmed.

Discussion : Immunologic and Syndrome Affecting Multiple Endocrine Organs:

When immune dysfunction affects two or more endocrine glands and other non-endocrine immune disorder are present, the polyglandular autoimmune syndrome should be considered.

Two main types: Type I : starts in childhood and is characterized by mucocutaneous candidiasis, hypoparathyroidism, adrenal insufficiency. Type II : (Schmidt syndrome) : more likely in adults, comprises adrenal insuffiency, thyroiditis and diabetes mellitus.

Features of Polyglandular Autoimmune (PGA) Syndrome :

 
 
5. A Case of Partial Mole
  Anita Kamat, Bhavesh Patel, Shashi Goyal, PB Paidhunghat
 

Vesicular mole is a relatively common condition to come across in obstetric practice; however one with size of 32 weeks is extremely rare.

Case History : A 25 year old patient Mrs. XYZ Gravida 3 Para 2 Living 2 with 14 weeks of gestation presented with bleeding and passing vesicles per vaginum since 1 1/2 months. On presentation, patient had low grade fever and anaemic with Hb of 7 gm% with serum bhCG of 18 lakhs iu/l and on clinical examination uterine size was 32 weeks. Patient underwent emergency suction evacuation under course pre operative antibiotics and 4 bottles of blood transfusion. Repeat suction evacuation was performed on day 3. In view of bhCG of 35,000 iu/l after 1st suction evacuation.
Histopathological report confirmed the diagnosis of partial mole. Postoperatively 4 doses of inj. Methotrexate were given alternating with Folinic acid. Patient was asked to follow up with bhCG titres of after 6 months.

Discussion : Hydatidiform mole/Vesicular mole is an abnormal condition of ovum where there are partly degenerative and partly hyperplastic changes in young chromosomic villi. It can be complete/partial and the aetiology is attributed to cytogenic abnormality, the complete mole having 46XX Karyotype and partial having triploid Karyotype 69XXY. It is principally a disease of chorion presenting with vaginal bleeding most commonly expulsion of grape like vesicles per vaginum and diagnostic sonography shows ‘snow-storm’ appearance while quantitative estimation of hCG is Gold standard and investigation. Management being essentially surgical.

 
6. An Extremely Rare Case of Biotinidase Deficiency in Both Parents
  Deepak Bhenki, Bhavesh Patel, BN Apte, S Goyal, PB PaiDhungat
 

A case of baby affected with biotinidase deficiency is extremely rare with frequency of 1 in 60,000 birth however there is no case reported where husband wife both are deficit in enzyme and wife has conceived.

Case History : A 30 yr patient married since 4 yr referred to our OPD for genetic counselling. She was primigravida with 12 week pregnant. The patient and her husband both are congenitally deaf and dumb since childhood. Pt had positive family history of all members being affected by similar ailment. There is no history on husband side. We referred to geneticist.

The investigation of both showed similar deficiency of enzyme biotinidase and patient advised termination of pregnancy by geneticist. Pregnancy was terminated with misoprostol tablet per vaginally and check curettage done for completeness of procedure.

Discussion : Biotin a water soluble vitamin of B-complex group, prosthetic group in 4 carboxylase enzyme. Two defects in cycle of biotin utilization results in multiple carboxylase deficiency Holocarboxylase synthetase deficiency also known as early onset (neonatal) multiple carboxylase deficiency is disorder of biotinylation. Other Biotinase deficiency also known as late onset (juvenile) multiple carboxylase deficiency, is a disorder of biotin recycling. Patient with Biotinidase deficiency the age of onset of symptoms range several weeks to several years of age, median and mean age of onset are 3 months and 5 and half. The organic aciduria commonly manifests as elevated concentrations of B-methylcrotonylglycine, B-hydrxyisovalerate, lactate, B-hydroxypropionate, and methylcitrate.

 
7. Pregnancy with Antiphospholipid Antibody Syndrome with Hypertension
  Darshana Vyas, SK Desai
 

Background : Antiphospholipid antibody syndrome is a relatively common condition causing repeated pregnancy loss in a patient accounting for 3-5% of all causes of recurrent pregnancy losses.

Case History : A 35 year old patient Mrs. S, G2, A1, a case of secondary infertility, conceived on ovulation induction and intrauterine insemination. She conceived for 1st time 10 years after marriage spontaneously. Pregnancy was terminated at 26 weeks of gestation in view of severe preeclampsia and reversal of blood flow to umbilical arteries in colour Doppler.

On investigations, she was diagnosed to have Antiphospholipid antibody syndrome. Lupus anticoagulant - positive, Anticardiolipin antibody IGM - strongly positive.

During her present gestation patient was started on T. ASA and low molecular weight heparin from 6.1 weeks of gestation. However in view of preeclampsia and normal colour doppler at 37 weeks of gestation, elective lower segment caesarian section was performed and delivered a Male child of 2.8 kg. Post delivery mother and baby well stable. Patient was restarted on low molecular weight heparin 12 hours after surgery and switched over to T. Warfarrin.

Discussion : In pregnancy phospholipid act like a sort of glue holds the dividing cells together and are necessary for growth of Placenta into the wall of uterus. Antiphospholipid can be IgG, IgA or IgM.

Adverse pregnancy outcomes : Recurrent early pregnancy losses, episodes of venous and arterial thrombosis, severe pre-eclampsia of early onset, severe IUGR.

The salvage rate may not always as satisfactory as desirable. Appropriate investigations and selection of drug would be the final determining factor for the good outcome.

 

MEDICALLY CONCEIVED TWINS HAVE AN INCREASE RISK OF PRETERM BIRTH

Compared with naturally conceived twins, twins resulting from subfertility treatment have a slightly decreased gestational age at birth and an increased risk of preterm birth. Veerstraelen and colleagues carried out a population based cohort study of more than 4300 twins pairs and found an odds ratio of 1.6 (95% CI 1.4 to 1.8) for preterm birth after artificial induction of ovulation. The risk is however limited to mild preterm birth, which is mainly due to dizygotic twinning after subfertility treatment.

BMJ, 2005 : 331 : 1173.

 
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