Akhter, J

Qureshi, R

English

eCommons@AKU

eCommons@AKUSection of Internal Medicine Department of MedicineJanuary 1995Partial lipodystrophy and successful pregnancyoutcomeJ AkhterAga Khan UniversityR QureshiAga Khan UniversityFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_med_intern_medRecommended CitationAkhter, J., Qureshi, R. (1995). Partial lipodystrophy and successful pregnancy outcome. Journal of Pakistan Medical Association, 45(1),24.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_intern_med/81Partial Lipodystrophy and Successful Pregnancy OutcomePages with reference to book, From 24 To 24 Jaweed Akhter ( Departments of Medicine and Obstetrics, The Aga Khan University Hospital, Karachi. ) R. Qureshi ( Departments of Gynaecology, The Aga Khan University Hospital, Karachi. )Partial lipodystrophy is a rare disorder, characterisedby lipoatrophy and insulin resistant diabetes1.Other features include hyperlipidenna, acanthosis nigrans, hepatomegaly and mesangiocapilaiyglomerulonephritis. Successful outcome of pregnancy is uncommon. We report a case of favourableoutcome in a female with partial lipodystrophy.Case report A 31 year old female presented to the ante-natal clinic at 9 weeks of her pregnancy. She was diagnosedas having diabetes mellitus, 5 years previously and was on oral hypoglycemic agents. She also hadhyperlipidemia on previous laboratoiy investigations. She conceived soon after her marriage. Therewas no family history of diabetes or other abnonnalities. When seen initially, she had almost completeloss of subcutaneous fat over her face, upper limbs and trunk. She had well developed musculature andmild facial hirsutism. She had 3 hyperpigmented areas on her trunk. There were no xanthomas orxanthelasma. Her BP was 120/80 and other cardiovascular examination was normal. There was nohepatomegaly. Initial ultrasound showed a single fetus at 9 weeks gestation. Her initial investigationsshowed Hb 13.4 gm/dl, HDL cholesterol 0.64 mmol1/1, cholesterol 7.61 mmolIl and triglycerides 7.10mmol/l, fasting blood glucose 11.9 mmol/dl random blood glucose 15.7 mmol/dl. Her renal and liverfunctions, serum testosterone and urinalysis were normal. She was started on insulin twice daily. Herrepeat ultrasound examination at 16 and 29 weeks was normal. Her total weight gain during pregnancywas 8 kg. During pregnancy her insulin requirement increased gradually till she was receiving 96 unitsdaily in two divided doses. She was monitored closely and her pregnancy was uneventful till 38th weekwhen she suddenly developed preeclampsia with BP 190/110 mmHg, pedal oedema and proteinuria.Ultrasound of fetus showed discrepancy between biparietal diameter and fetal length consistent with 32weeks and 36 weeks respectively. She was admitted and blood pressure was controlled withintravenous hydralazine, following which she underwent caesarian section and delivered a normal babyof 3.5 kg weight. Immediately following delivery her insulin requirement dropped to 16 units in 24hours and her blood pressure was controlled without treatment.Discussion Partial lipodystrophy is characterized by lipoatrophy and insulin resistant diabetes. Apart from ketosisresistant diabetes and total loss of body adipose tissue, other features include hyperlipidemia,hepatomegaly, acanthosis nigrans, mesangiocapillary glomerulonephritis and increased basal metabolicrate with normal thyroid function2. Partial lipodystrophy is more common with loss of adipose tissue inparts of the body and associated with some or all the above features. Certain types of partial lipodystrophy are inherited as autosomal dominant traits3,4. The loss of adipose tissue in these formsusually spares the face. The most common type of partial lipodystrophy is progressive orcephalothoracic lipodystrophy resulting in a progressive loss of subcutaneous fat in the upper half ofthe body including face. This disorder is not inherited and affects women more than men5. Pregnanciesin women with partial lipodystrophy carry a high risk of intrauterine death6,7. Intrauterine growthretardation is also seen in these cases and the exact cause not known but most occur in the thirdtrimester of pregnancy. Two case reports of successful outcome of pregnancy have also been reported8.The patient we described had features consistent with partial lipodystrophy loss of subcutaneous fatover her face and upper trunk, in association with diabetes mellitus and hyperlipidemia. Her historyindicated that the onset of this disease was in early adult life. She had a non-eventful pregnancy until38 weeks, when she developed significant pre-eclampsia with ultrasound evidence of growthretardation. Her outcome as described was successful. As most patients described in the literaturedevelop problems late in third trimester as did our patient, we feel early elective delivery should beadvocated for these patients. Partial lipodystrophy may be associated with successful pregnancyoutcome but these patients require very close monitoring.References 1. Robbins, D.C. and Tager, H.S. Mutant insulins and lipotrophic diabetes: An emerging genetic basisfor certain cases of diabetes. De Groot ed. Endocrinology. 2nd ed. Philadelphia, WB Saunders,1989;pp.1400-1407.2. Senior, B. and Gellis, S.S. The syndromes of total lipodystrophy and partial lipodystrophy.Paediatrics, 1964;33 :543-97.3. Dunnigan, M., Cochrane. M., Kelly, A. et al. Familial lipotrophic diabetes with dominanttransmission. Q.J. Med., 1974;169:33-36.4. Kobberling, J., Willms, B., Kattermann, R. et al. Lipodystrophy of the extremities: a dominantlyinherited syndrome associated with lipotrophic diabetes. Hum. Genet., 1 975;29: 111-15.5. Piscatelli, R.L., Vieweg, W.V.R., Havel, R.J. Partial lipodystrophy. Metabolic studies in threepatients. Ann. Intern. Med., 1970;73:963-70.6. Fitch, N. and Tulandi, T. Progressive partial lipodystrophy and third-trimester intrauterinefetal death,Am. J. Obstet. Gynaecol., 1 987;1 56:1195-96.7. Haxton, M.J. Progressive partial lipodystrophy in association with intrauterine death andgrowthretardation. Am. 7. Obstet. Gynaecol., 1983;147:837-38.8. Catalano, P.M, Capeless, EL.. Simmons, G.M. et al. Successful pregnancy outcome in associationwith lipotrophic diabetes mellitus. Obstet. Gynaecol., 1990;76:978-79.

Partial lipodystrophy and successful pregnancy outcome

https://ecommons.aku.edu/cgi/viewcontent.cgi?article=1080&amp;context=pakistan_fhs_mc_med_intern_med

Partial lipodystrophy and successful pregnancy outcome

Abstract

Similar works

Full text

Available Versions

eCommons@AKU