RM-493 Treatment Trial in Proopiomelanocortin (POMC) Deficient Patients

Inclusion Criteria: * Written informed consent * Homozygous or compound heterozygous (different gene mutation on both alleles) POMC, LEPR or PCSK1 gene mutation * Obesity (BMI \> 30 kg/m2; + 2 BMI SDS) * No other therapeutic option, which might cure the patient (e.g. bariatric surgery (see chapter 8)) * Negative Pregnancy test * Highly effective contraception in women (defined as pearl index \< 1), if necessary also for partners of test persons) * No participation in other clinical trials according to AMG (Arzneimittelgesetz) (2 months before and after) at the time of this trial * Normal or minimally elevated blood pressure (measured in 24RR monitoring or similar methods) according the guidelines of the ESH (European Society of Hypertension) and Deutsche Hochdruckliga: systolic \> 159 mmHg/diastolic 99 mmHg * sufficient kidney and liver function (Creatinine, ALT, AST) * normal values Alanine-Aminotransferase (ALT) (female): \< 31 U/l * normal values Alanine-Aminotransferase (ALT) (male): \< 41 U/l * normal values Aspartate-Aminotransferase (AST) (female \> 17 years): \< 35 U/l; (female \< 17 years): 16- 46 U/l * normal values Aspartate-Aminotransferase (AST) (male \> 17 years): \< 50 U/l; (male \< 17 years): 16-46 U/l * normal values bilirubins (male and female) up to 1,2 mg/dl * normal values Creatinine (female \> 15 years): 0,51-0,95 mg/dl) ; (female \< 15 years): 0,46-0,77 mg/dl * normal values Creatinine (male \> 15 years): 0,67 - 1,17 mg/dl) ; (male \< 15 years): 0,46-0,77 mg/dl Exclusion Criteria: * Pregnancy or Breastfeeding * All contraindications against study medication (including auxiliary substances) * Interactions with study medication * Participation of the patient in a clinical study within the last 2 months * Intolerance against albumin * Concomitant diseases, impaired organ functions, except for known, concurrent GI disorders or other clinical findings expected in PCSK1 or LEPR gene disorders * Renal insufficiency (Creatinine \> 0,95 mg/dl (female), \> 1,17 mg/dl (male)) * Impaired liver function (Bilirubins \> 1.2 mg/dl) * Neurological / psychiatric diseases * HIV Infection * Active Hepatitis B or C * Melanoma or Melanoma occurrence in the family history * Non-compliance * Subjects who are legally detained in an official institution