Congenital glucose galactose malabsorption complicated with rickets and nephrogenic diabetes insipidus

Congenital Glucose Galactose malabsorption [CGGM] is a rare disorder with limited data from the Arab world. We report the first case of CGGM in Oman. B.S.A two years old female who presented with chronic osmotic diarrhea since birth with hypernatraemic dehydration. B.S was found to have Glucose Galactose Malabsorption based on clinical trial of ORS and elemental formula. Symptoms resolved on introduction of Carbohydrate free formula. The patient developed many complications while on TPN including rickets and nephrogenic diabetes insipidus. These complications have not been reported earlier in CGGM

A case of nephrogenic diabetes insipidus caused by obstructive uropathy due to prostate cancer

Nephrogenic diabetes insipidus (DI) secondary to chronic urinary tract obstruction is a rare disease. The exact cause is unknown but it is likely that increased collecting duct pressures cause damage to the tubular epithelium, resulting in insensitivity to the action of arginine-vasopressin (AVP). A 77-year-old man complaining of polyuria and polydipsia was treated with alpha glucosidase inhibitor under the impression of polyuria due to diabetes mellitus. But his symptoms did not improve. Water deprivation and AVP administration study revealed that the patient had nephrogenic DI. Urinary tract obstruction due to an enlarged prostate was suggested as a principal cause of nephrogenic DI. The patient underwent transurethral resection of the prostate and bilateral subcapsular orchiectomy. After surgery, the urine osmolarity was normalized and the patient became symptom-free. We report a case of nephrogenic DI due to obstructive uropathy which was cured by surgery eliminating obstruction.

Síndrome nefrótico complicado con trombosis cerebral del seno venoso longitudinal superior, diabetes insípida neurogénica e intoxicación por fenotiazinas / Cerebral longitudinal sinus thrombosis, neurogenic diabetes, and phenotiazine intoxication in a patient with nephrotic syndrome

Introducción. Es característico del síndrome nefrótico (SN) la presencia de recaídas, aún en niños que inicialmente respondieron satisfactoriamente con prednisona y que presentaron remisión completa. Muchos de estos pacientes viven en condiciones que retardan el inicio del tratamiento o provocan la aparición de complicaciones: peritonitis, sepsis, celulitis, neumonía o trombosis venosas. Estas últimas generalmente localizada en la vena renal, pero puede presentarse en cuanquier sitio anatómico. Caso clínico. Se presenta el caso de un niño de años de edad, que durante una recaída de SN desarrolló una trombosis cerebral en el seno venoso longitudinal superior, además de diabetes insípida de origen central, así como intoxicación por fenotiazinas, que inicialmente fue causada de varias manifestaciones extrapiramidales. Se analiza la evolución de la trombosis y de la diabetes insípida, señalando la resolución favorable que se obtuvo con su manejo conservador. Conclusión. Se destaca la importancia de efectuar un estudio de tomografía axial computada de cráneo en los niños que durante una recaída de SN presentan deshidratación con cefalea o crisis convulsivas